Peds EOR Cardiology

What type of murmur: Patent ductus arteriosus

Continuous murmur

Surgical delamination of the tricuspid valve septal leaflet. The mobilized leaflet is then rotated and reattached at the true atrioventricular junction, followed by leaflet-to-leaflet attachment, creating a "leaflet cone." Subsequently, the atrialized portion of the right ventricle is plicated and patent foramen ovale is repaired.

Cone repair of Ebstein anomaly

What congenital infection is associated with patent ductus arteriosus?

Congenital rubella

_____________ is the congenital infection associated with patent ductus arteriosus.

Congenital rubella

Hypertrophic cardiomyopathy can be associated with ___________________, an autosomal recessive inherited disease that causes progressive damage to the nervous system and manifests initially with gait disturbance, but does not affect cognitive function.

Friedreich's ataxia

Functional or pathological murmur? Peripheral pulmonary stenosis in infant (0-8 months)

Functional murmur

What test can be used to rule out pulmonary causes vs blood circulation cause - R to L shunt?

Hyperoxia Test

What heart defect? Atresia, stenosis or hypoplasia of aortic valve or mitral valve and hypoplasia of aorta and arch

Hypoplastic Left Heart Syndrome

What heart sound? Crunching "leather"

rubs

AV Septal Defect (AVSD) tx

surgical mgmt

What is V in PROV for Tetrology of Fallot?

ventricular septal defect

What are the Three cardiac changes that can occur in the normal Neonate?

Transitional circulation in series Patent Ductus Arteriosus closure Fall in Pulmonary Vascular Resistance

When infants present with cyanosis, what noncardiac causes must be ruled out?

Sepsis Pulmonary Hypoglycemia Dehydration Hypoadrenalism Metabolic disorders

Which adaptation of fetal circulation disappears *within minutes* after birth?

Foreman Ovale

The severity of the manifestations of tetralogy of Fallot is mainly dependent on the degree of _______________.

pulmonary stenosis

What is P in PROV for Tetrology of Fallot?

pulmonary stenosis

Patent ductus arteriosus causes a (systolic/diastolic/continuous) murmur.

continuous

You can differentiate VSD from PDA because the murmur of a patent ductus arteriosus is ______________, not holosystolic. The murmur accentuates in late systole.

continuous

What is the tetrad of "PROV" in TOF?

1. Pulmonary stenosis 2. Right ventricular hypertrophy 3. Overriding aorta 4. Ventricular septal defect

DDx Cyanotic CHD

1) Tetrology of Fallot 2) Transposition of Great Arteries 3) Tricuspid Atresia 4) Pulmonary Atresia 5) Total Anomalous Pulmonary Venous Return 6) Truncus Arteriosus 7) Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart Syndrome Repair stages? other tx option?

1st stage Norwood -Neonate -Most difficult -Arch reconstruction -BT shunt 2nd stage Glenn 3rd stage Fontan Transplant

Cyanosis is obvious when O2 sat is:

< 85%

The ___________ valve is the valve most commonly involved in acute rheumatic fever.

mitral

What heart defect? Trisomy 21 (Down's) ASD, VSD physiology Volume load to RV & LV AV valve regurgitation Holosystolic murmur Cardiomegaly CHF

AV Septal Defect (AVSD)

How to Recognize a Suspected Cardiac Defect in a Neonate during the Physical Exam?

Abnormal heart rate Comfortable tachypnea Abnormal heart sounds Abnormal precordial activity Abnormal O2 saturation and gradient BP gradient Abnormal femoral pulses Hepatomegaly

A patent ductus arteriosus is what kind of defect?

Acyanotic left-to-right shunt

A ventricular septal defect is what kind of defect?

Acyanotic left-to-right shunt

An atrial septal defect is what kind of defect?

Acyanotic left-to-right shunt

Aortic stenosis is what type of defect?

Acyanotic outflow obstruction

Coarctation of aorta is what kind of defect?

Acyanotic outflow obstruction

Pulmonary stenosis is what type of defect?

Acyanotic outflow obstruction

________________ are reactive histiocytes with wavy, slender, caterpillar-like nuclei seen in Aschoff bodies of acute rheumatic fever.

Anitschkow cells

Syndrome and heart defect:: 22q11 (De George)

Aortic arch Conotruncal

What heart defect? Usually bicuspid valve Systolic ejection murmur Low cardiac output that results in: -Poor perfusion -Respiratory distress -Mild cyanosis

Aortic stenosis

_______________ are foci of chronic inflammation seen histologically in the myocarditis of acute rheumatic fever.

Aschoff bodies

For acute rheumatic fever _______________controls fever and pain caused by arthritis and carditis.

Aspirin

Treatment summary for acute rheumatic fever

Aspirin or another NSAID Sometimes corticosteroids Antibiotics

EKG shows right bundle branch block. What type of heart defect do you suspect?

Atrial Septal Defect

Frade II-III/VI systolic ejection murmur at left 2nd or 3rd intercostal space

Atrial Septal Defect

Widely split and fixed S2 into A2

Atrial Septal Defect

Definitive treatment for Transposition of the Great Arteries

Balloon atrial septostomy

Tx of pulmonary stenosis

Balloon valvuloplasty Surgical repair

What is the treatment of pulmonary stenosis?

Balloon valvuloplasty Surgical repair

tx for aortic stenosis?

Balloon valvuloplasty Surgical repair

________________ is the type of penicillin used for prophylaxis against rheumatic fever and rheumatic heart disease.

Benzathine penicillin G

___________ aortic valve is a cardiac valve defect associated with coarctation of the aorta.

Bicuspid

Blood flow for Pulmonary Valve Atresia with Intact Septum

Blood exits RV either back through RA or connections between RV and coronary artery circulation. Often PFO allows for right to left shunting to systemic circulation.

Fetal Cardiology tx

Catheter Surgical Genetic

What heart sounds? Higher pitched Sharp/crisp

Clickes

A 24-hour-old girl is found to be *cyanotic* in the newborn nursery. She was born by spontaneous vaginal delivery at 38 weeks to a gravida 1, para 0 healthy mother who received routine prenatal care. She is *small for her gestational age*. She has *lower extremity cyanosis* along with a *mesh-like mass on the back of her neck.* Her pulse is 150/min, respirations are 48/min, and a blood pressure is 120/80 mm Hg in the right arm, 124/82 mm Hg in the left arm, 80/40 mm Hg in the right leg, and 85/45 mm Hg in the left leg. *Femoral pulses are 1+ and delayed.* Cardiac examination shows a *continuous murmur in the interscapular area*. Faint *crackles are heard at the bases of the lung fields bilaterally* on auscultation. What is the most likely diagnosis?

Coarctation of the Aorta

Congenital heart defect defined as a narrowing of a segment of the aorta.

Coarctation of the Aorta

What heart defect? Associated AOV, MV Systolic murmur at back

Coarctation of the Aorta

A young boy comes into your office. He has elevated blood pressure when taken in his arm, but no femoral pulses can be palpated. What is the most likely diagnosis?

Coarctation of the aorta

An 8-year-old boy is seen for the first time in your office. His parents report that he tires easily and often complains of weakness in his legs. Physical exam shows a healthy boy with a blood pressure of 141/91 mmHg. You notice that his lower extremities are slightly atrophic with a mottling appearance. Upon further examination, he is found to have very weak and delayed femoral pulses with a blood pressure of 96/60 in the lower extremities. He has a late systolic ejection murmur on cardiac auscultation. CXR is performed demonstrating a "figure of 3 sign." What is the most likely diagnosis?

Coarctation of the aorta

Female patients diagnosed with ____________________ should have a karyotype analysis to rule out Turner syndrome.

Coarctation of the aorta

________________________ is a congenital heart defect defined as a narrowing of a segment of the aorta.

Coarctation of the aorta

Chest X-ray of a patient with coarctation of the aorta will show __________________, dilated left subclavian artery and post-stenotic aortic dilation.

notching of ribs

Congenital heart condition in which your heart points toward the right side of your chest instead of the left side.

Dextrocardia

Persistent truncus arteriosus is associated with __________________ which is caused by 22q:11.2 deletion .

DiGeorge syndrome

What is a hereditary T-cell disorder that is associated with tetralogy of Fallot?

DiGeorge syndrome

Tetralogy of Fallot is associated with what syndromes?

DiGeorge syndrome Down's syndrome

______________is contraindicated in patients with hypertrophic cardiomyopathy as the outflow tract obstruction can worsen following its administration.

Digoxin

What is critical for newborns with Transpositions of the great arteries until surgery?

Ductal/ ASD mixing

Which adaptation of fetal circulation disappears *within hours* after birth?

Ductus Arteriosus Umbilical Artery

What type of murmur: Aortic regurgitation

Early diastolic murmur

What type of murmur: Pulmonic regurgitation

Early diastolic murmur

Malformed and apically displaced tricuspid valve

Ebstein's Anomaly

For hypertrophic cardiomyopathy ______________is the key to diagnosis - will show left ventricular hypertrophy with a thickened septum, small left ventricle, and diastolic dysfunctio

Echocardiogram

What diagostic tool? Ultrasound High resolution images in infants and children Multiple views Comprehensive Non-invasive Limitations

Echocardiogram

What is the best diagnostic tool to confirm the diagnosis of Tetralogy of Fallot?

Echocardiography

What is the best diagnostic tool to confirm the diagnosis of transposition of the great vessels?

Echocardiography

What is used to make a definitive diagnosis of an atrial septal defect?

Echocardiography

_______________ is used for confirming the diagnosis of patent ductus arteriosus.

Echocardiography

__________________ is an annular, non-pruritic rash with erythematous borders seen in acute rheumatic fever; it is typically found at the trunk and limbs.

Erythema marginatum

Congenital Heart Disease: risk factors

Family history Multiple fetuses Genetic syndromes

What fetal cardiac surgery option? Congential heart block

Fetal Cardiac Pacing

Diverts venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries without passing through the morphologic right ventricle.

Fontan Procedure

What procedure? 3rd stage for single ventricle hearts 2 years of age Oxygen saturation normal

Fontan Procedure

What procedure? 2nd stage for single ventricle hearts (left or right) Done at 6 months of age Blue blood directly to lungs, "skipping" the heart Oxygen saturation 85%

Glenn Procedure -Superior vena cava to pulmonary artery

What grade murmur? Barely audible

Grade I

What grade murmur Clearly audible

Grade II

What grade murmur moderately loud

Grade III

What grade murmur loud/thrill

Grade IV

What grade murmur? loud/thrill with stethoscope just off chest

Grade V

What grade murmur? very loud/visible thrill

Grade VI

Acute rheumatic fever is an autoimmune complication of pharyngitis caused by ______________________.

Group A streptococcus

Things to look at on a CXR

Heart position -Dextrocardia Heart size Pulmonary vascularity Edema Pulmonary pathology

The classic clinical sign of coarctation of the aorta is:

Higher blood pressure in the arms than in the legs and pulses that are bounding in the arms but decreased in the legs.

Type ___hypersensitivity is seen in rheumatic fever.

II

Suspect Congenital Heart Disease if there is:

Incorrect transitional circulation: -Parallel instead of in Series Obstruction of series circulation and PDA closes Fall in Pulmonary Vascular Resistance and shunting/mixing occurs

_______________ is the treatment for patent ductus arteriosus, because it decreases prostaglandin E1/E2 production.

Indomethacin

ECG is a good test for:

Ischemia Heart injury Electrolytes

What fetal cardiac surgery option? For severe Aortic Stenosis that may lead to irreversible LV dysfunstion.

Intrauterine Balloon Aortic Valvuloplasty

What fetal cardiac surgery option? For severe Pulmonic Stenosis seen in Right Ventricular and Tricuspid Valve hypoplasia.

Intrauterine Balloon Pulmonary Valvuloplasty

What fetal cardiac surgery option? When the existence of an interatrial communication is important for oxygenated blood to be distributed to the body in Hypoplastic Left Heart syndrome

Intrauterine intervention on Atrial Septum.

Rheumatic fever major criteria: J♥NES

Joints - polyarthritis ♥ - carditis Nodules (subcutaneous) Erythema marginatum Sydenham's chorea

Coarctation of the aorta will show what kind of murmur?

Late systolic ejection murmur

What type of murmur: Mitral valve prolapse

Late systolic murmur

Pulmonary veins normally connect to..

Left atrium

EKG finding for Primum atrial septal defect?

Left axis deviation

What type of murmur: Mitral stenosis

Mid-diastolic murmur

What type of murmur: Tricuspid stenosis

Mid-diastolic murmur

_________________ is a complication of acute rheumatic fever characterized by swelling and pain in large joints that resolves, and then migrates to other large joints.

Migratory polyarthritis

What degree of Coarctation of the Aorta? Hypertension Differential pulses, BP

Mild

Suspect Congenital Heart Disease if there is:

Murmur Arrhythmia Congestive Heart Failure Cyanosis

_________________ is the most common cause of death during the acute phase of rheumatic fever.

Myocarditis

What medications can put a fetus at risk for congenital heart disease?

NSAIDs ACE inhibitors Retinoic acid Thalidomide Phenytoin Lithium

What are the maternal risk factors for congenital heart disease?

Obesity Diabetes Epilepsy Hypertension Preeclampsia Thyroid disorders PKU Mood disorders Connective Tissue Disease Advanced Age>40 Alcohol/Tobacco use

How to conduct a hyperoxia test?

Obtain Room Air ABG from right radial artery Provide 100% O2 for 10 minutes Obtain ABG from right radial artery -pO2 >100. pulmonary disease likely. -pO2 < 70. Increases <30. cardiac cause likely (R-L shunt)

ECG of a neonate with tricuspid atresia shows increased height of the _______, indicating atrial enlargement.

P wave

What type of murmur: Mitral regurgitation

Pansystolic, Holosystolic, or Reguritant murmur

What type of murmur: Tricuspid stenosis

Pansystolic, Holosystolic, or Reguritant murmur

What type of murmur: Ventricular septal defect

Pansystolic, Holosystolic, or Reguritant murmur

Widened pulse pressure with low DBP

Patent ductus arteriosus

Neonates with tricuspid atresia should be given ___________ to keep the ductus arteriosus open.

Prostaglandin E1

Surgical repair is the mainstay of treatment for hypoplastic left heart syndrome, with (drug) _______________ used to keep the patent ductus arteriosus open.

Prostaglandin E1

What can be given to patients with transposition of the great vessels to maintain a patent ductus arteriosus until a surgical correction is established?

Prostaglandin E1

What needs to be given emergently to do keep ductal/ASD mixing in newborns with transposition of the great arteries?

Prostaglandin E1

_____________ can be given to patients with transposition of the great vessels to maintain a patent ductus arteriosus until a surgical correction is established.

Prostaglandin E1

____________________ should be administered to neonates with aortic coarctation to keep the ductus arteriosus open.

Prostaglandin E1

What CHD? Complete obstruction to right ventricular outflow with varying degrees of right ventricular and tricuspid valve atresia Ductal dependent. Closure of PDA results in rapid deterioration.

Pulmonary Valve Atresia with Intact Septum

What heart defect? Variable systolic ejection murmur Right Ventricular hypertrophy CXR normal

Pulmonary stenosis

DDx for acyanotic outflow obstruction

Pulmonary stenosis Aortic stenosis Coarctation of aorta

What is the compensation of Hypoplastic Left Heart Syndrome?

Results in Right ventricle supporting both pulmonary and systemic circulation.

A 130-year-old girls presents with 2 weeks of arthralgias, migrating from distal to proximal joints. It began with increased warmth and erythema in her right ankle and left knee. She has a low-grade fever and reports a history of sore throat and swollen glands about 1 month ago. On physical exam she has red skin lesions on the trunk and proximal extremities, and also small, non-tender lumps located over the joints. Antistreptolysin O titer is positive. What is the most likely diagnosis?

Rheumatic Fever

EKG finding for Secundum atrial septal defect?

Right axis deviation

An _____ heart sound is commonly associated with hypertrophic cardiomyopathy.

S4

Ostium (primum/secundum) atrial septal defects are the most common type of atrial septal defects in adults.

Secundum

What is the most common type of atrial septal defect?

Secundum

What are the 3 types of Atrial septal defect?

Secundum (70%) Primum (15-20%) Sinus Venosus

What is a compensatory mechanism that child with a right to left shunt (TOF) use during exercising?

Squatting

Functional murmurs in a child 2-10 yrs old?

Still's Pulmonary flow murmur Venous hum Subclavian bruit

Syndrome and heart defect: williams

Supervalvar Aortic stenosis Pulmonic Stenosis

Patent ductus arteriosus treatment?

Surgery Catheter device

tx of Coarctation of the Aorta

Surgery balloon dilation

What is the definitive treatment for tetralogy of Fallot?

Surgical correction

_________________is a neurological disorder that appears in acute rheumatic fever; it is characterized by rapid, involuntary movements of the face, hands and feet.

Sydenham chorea

Rheumatic Fever is caused by an acute, inflammatory immune response to Group A Strep, with the formation of antistreptolysin antibodies which react with proteins on the:

Synovium Heart muscle Heart valves

What type of murmur: Aortic stenosis

Systolic ejection murmur

What type of murmur: Atrial septal defect

Systolic ejection murmur

What type of murmur: Pulmonic stenosis

Systolic ejection murmur

Tetralogy of Fallot is characterized by the presence of a (systolic/diastolic) murmur.

Systolic murmur

Ductus dependent pulmonary circulation

TOF with PA pulmonary atresia with intact Interventricular septum critical PS Tricuspid Atresia with PS/PA Single ventricle with PS/PA Severe Ebstein anomaly Complete TGA with intact IVS

Pulmonary blood flow in Ebsterin's anomaly

TR Inadequate RV RVOT obstruction/hypoplasia RV failure

A 2-week-old newborn brought to the ER by his mom who reports a sudden loss of conciseness during feeding and with crying. She also has noticed that the infants lips have turned blue on three occasions during feeding. blood pressure is 75/45 mmHg, a pulse is 170/min, and respirations are 44/min. A grade 3/6 harsh systolic ejection murmur is heard at the left upper sternal border. A CXR shows a small boot shaped heart and decreased pulmonary vascular markings. What is the most likely diagnosis?

Tetralogy of Fallot

A 3-year-old boy presents to his cardiologist for routine follow-up for his congenital heart disease. He was diagnosed in infancy when he presented with *bluish skin shortly after birth*. During the current visit, he does not appear to be in distress. A *harsh systolic ejection murmur* is heard over the *left mid-sternal border* upon auscultation, and no S2 split can be appreciated. There is no jugular vein distention, and his liver is not enlarged. His parents say that he has *episodes of weakness and paleness* several time a day, during which *squatting improves his symptoms*. What is the most likely diagnosis?

Tetralogy of Fallot

What do you suspect? Associated with DiGeorge syndrome (22q11.2 deletion syndrome) and Down's syndrome. Variable cyanosis and murmur as newborn -85 -95% -Ductal dependent CXR -Decreased markings

Tetralogy of Fallot

Syndrome and heart defect: Charge

Tetralogy of Fallot Conotruncal

Cyanosis may be seen in several types of CHD such as?

Tetrology of Fallot Transposition of Great Arteries Pulmonary Atresia Ebstein's Anomaly Truncus Arteriosus Hypoplastic Left Heart Syndrome Total Anomalous Pulmonary Venous Return

A female newborn is born at 34 weeks gestation and develops *cyanosis of lips and tongue 12 hours after birth*. Her mother says that she is *harder to wake* and has been very *inactive*. Physical examination shows *perioral cyanosis* and a *capillary refill time of 4 seconds*. Cardiac examination shows regular rate and rhythm, but a *loud, fixed-split S2*, a *III/VI systolic ejection murmur* best appreciated *at the left upper sternal border,* and a I/VI *diastolic rumble. Hepatomegaly* is also appreciated on examination. Which of the following is the most likely diagnosis?

Total Anomalous Pulmonary Venous Return (TAPVR)

A 23-hour-old infant delivered vaginally at 38 weeks gestation was born with progressive *cyanosis in absence of respiratory distress*. Physical examination shows *central cyanosis,* but is otherwise normal. The patient's delivery was smooth without any complications. Subcostal echocardiogram shows *failure of the aorticopulmonary septum to spiral.* Which of the following conditions is most likely seen in the newborn's mother and is associated with this defect?

Transpositions of the great arteries

What CHD? Parallel circulations Severe cyanosis Increased pulmonary blood flow Ductal/ASD mixing

Transpositions of the great arteries

A 12-hour-old female newborn delivered at 30 weeks' gestation is found to be *tachypneic and lethargic*. The nurse on duty reports *generalized cyanosis over the limbs and trunk*. PE shows a *single, second heart sound that does not split* at any point. What is the most likely diagnosis?

Tricuspid Atresia

Congenital heart disease in which there is a complete absence of the tricuspid valve.

Tricuspid Atresia

What CHD? No communication between right atrium and right ventricle Small right ventricle Variable presentation ventricular septal defect present Blood exits the right atrium through obligate patent foramen ovale

Tricuspid Atresia

What congenital heart defect involves the absence of a tricuspid valve and a hypoplastic right ventricle as a result?

Tricuspid Atresia

What other congenital cardiac disorder is most commonly associated with an atrial septal defect?

Tricuspid atresia

What genetic syndromes most commonly have congenital heart disease?

Trisomy 21 Turner Syndrome DiGeorge deletion 22q

What type of ventricular septal defect: "sub- pulmonary" "infundibular"

Type 1

What type of ventricular septal defect: "peri-membranuous," "para-membranous"

Type 2

What type of ventricular septal defect: Typically occurs in Down syndrome. "Peri-inlet" "AV canal"

Type 3

What type of ventricular septal defect: Muscular defect

Type 4

Which adaptation of fetal circulation disappears *within days* after birth?

Umbilical vein Ductus venosus

5 Adaptations of fetal circulation

Umbilical vein Ductus venosus Foramen ovale Ductus Arteriosus Umbilical Artery

A false-positive (blood test) ___________ can be seen with rheumatic fever.

VDRL

The murmur associated with hypertrophic cardiomyopathy is worsened by conditions that cause reduced ventricular volume such as the _____________, sudden standing, and tachycardia.

Valsalva maneuver

A 4-year-old boy who is brought to your office by his parents because he gets tired very easily and cannot keep up with the other children. On exam, you hear a loud, harsh, holosystolic murmur at the left lower sternal border without radiation to the axillae. What is the most likely diagnosis?

Ventricular Septal Defect

A loud, harsh, holosystolic murmur at the lower left sternal border

Ventricular Septal Defect

Syndrome and heart defect: Vacterl

Ventricular Septal Defect

What defect? One of the most common congenital heart defects. Occurs in isolation or with other CHD (such as Down's) Presentation depends on size Left to right shunting LV output must increase to maintain systemic blood flow at normal levels

Ventricular Septal Defect

What is the most common cardiac congenital anomaly?

Ventricular Septal Defect

DDx for acyanotic left-to-right shunt

Ventricular Septal defect Patent ductus arteriosus Atrial Septal defect

Acyanotic Heart Diseases

Ventricular septal defect Atrial septal defect Patent ductus arteriosus Pulmonic stenosis Aoritic stenosis Coarctation of Aorta Atrioventricular septal defect

Syndrome and heart defect: Trisomy 13 & 18

Ventricular septal defect (VSD) Atrial septal defect (ASD) Patent ductus arteriosus (PDA)

______________ and diltiazem are non-dihydropyridine calcium channel blockers that can be used to treat hypertrophic cardiomyopathy.

Verapamil

What arrhythmia is associated with Ebstein's anomaly?

WPW syndrome

A 3-year-old boy who was recently diagnosed with a congenital heart defect, comes to his pediatrician's office for a follow-up visit. Two months ago, he was referred to a pediatric cardiologist after his parents noticed that he seemed to be *in distress* frequently, *especially after running around the house*. He would also *squat down to catch his breath*. The pediatric cardiologist informed the parents that the squatting helps to compress the femoral arteries which increases systemic blood pressure, reducing the right-to-left blood shunting in his heart. *Which of the following heart sounds is most likely to be auscultated in this patient due to his condition?*

Wide splitting of S2

Hypertrophic cardiomyopathy has a thick interventricular septum that is too close to the _____________, which causes outflow obstruction.

anterior mitral leaflet

Rheumatic Fever is caused by an acute, inflammatory immune response to Group A Strep, with the formation of _______________ which react with proteins on the synovium, heart muscle, and heart valves

antistreptolysin antibodies

Persistent truncus arteriosus is a congenital heart defect that involves failure of the truncus arteriosus to divide into the pulmonary trunk and aorta due to lack of ______________________ formation.

aorticopulmonary septum

Transposition of great vessels is due to the failure of the ____________________ to spiral during fetal development.

aorticopulmonary septum

Treatment of Coarctation of the aorta is ________________, or surgical correction.

balloon angioplasty with stent placement

A ____________shaped heart is seen on chest X-ray in a patient with tetralogy of Fallot.

boot

Cardiogenic Shock is suggested when______ is seen on CXR and volume resuscitation is unsuccessful

cardiomegaly

Tetralogy of Fallot is the most common cause of early childhood _____________.

cyanosis

The most common presentation of tricuspid atresia is ___________of the neonate.

cyanosis

The most common sign of hypoplastic left heart syndrome is __________.

cyanosis

The most common sign of transposition of the great vessels is _________of the neonate.

cyanosis

Hypoplastic Left Heart Syndrome is a (cyanotic/ acyanotic) congenital heart disorder.

cyanotic

Pulmonary Atresia is a (cyanotic/ acyanotic) congenital heart disorder.

cyanotic

Tetrology of Fallot is a (cyanotic/ acyanotic) congenital heart disorder.

cyanotic

Total Anomalous Pulmonary Venous Return is a (cyanotic/ acyanotic) congenital heart disorder.

cyanotic

Transposition of Great Arteries is a (cyanotic/ acyanotic) congenital heart disorder.

cyanotic

Tricuspid Atresia is a (cyanotic/ acyanotic) congenital heart disorder.

cyanotic

Truncus Arteriosus is a (cyanotic/ acyanotic) congenital heart disorder.

cyanotic

Infantile coarctation of the aorta is associated with both _________________ and Turner syndrome.

patent ductus arteriosus

Squatting (increases/decreases) the cyanosis in tetralogy of Fallot.

decreases

TOF is a cyanotic heart disorder that is characterized by four defects that together cause oxygen (rich / deficient) blood to flow out of the heart and into the rest of the body.

deficient

In coarctation of the aorta, there is a pulse ______ between the brachial and femoral pulses.

delay

Hypertrophic cardiomyopathy (HOCM) is a congenital or acquired disorder characterized by marked ventricular hypertrophy with (systolic / diastolic) dysfunction.

diastolic

Hypertrophic cardiomyopathy is classified as a (systolic/diastolic) dysfunction.

diastolic

Infants with a large VSD who develop congestive heart failure and growth retardation should be treated first with __________ and diuretics. If this fails, surgical intervention is necessary within the first 6 months of life.

digoxin

Verapamil and ____________ are non-dihydropyridine calcium channel blockers that can be used to treat hypertrophic cardiomyopathy.

diltiazem

Infantile coarctation of the aorta is located (distal/proximal) to the aortic arch.

distal

Tricuspid atresia is due to the failure of the _____________ of the AV canal to fuse.

endocardial cushions

In coarctation of the aorta, the aortic shadow shows "____________ sign" due to dilatation of the proximal and distal segments surrounding the coarctation.

figure of 3

The four features of tetralogy of Fallot are ____________, right ventricular hypertrophy, a ventricular septal defect, and an overriding aorta.

pulmonary stenosis

The murmur of TOF is classically described as a _____________________ murmur heard best at the left sternal border

harsh systolic ejection

Beta blockers, calcium channel blockers, or cessation of ________________ are all possible managements of hypertrophic cardiomyopathy.

high intensity athletics

Ductus dependent systemic circulation

hypoplastic left heart syndorme Crictical AS "Shone" complex variants Coarctation of aorta Interrupted Aortic Arch

Rheumatic fever is caused by an acute, inflammatory ________________ to Group A Strep, with the formation of antistreptolysin antibodies which react with proteins on the synovium, heart muscle, and heart valves

immune response

An atrial septal defect will cause (increased/decreased) oxygen saturation in the right side of the heart.

increased

Squatting (increases / decreases) the peripheral vascular resistance which diminishes the right-to-left shunt and increases pulmonary blood flow.

increases

Squatting increases the peripheral vascular resistance which diminishes the right-to-left shunt and (increases / decreases) pulmonary blood flow.

increases

Although rheumatic fever follows a streptococcal throat infection (strep throat), it is not an infection.. Rather, it is an _____________ reaction to the infection.

inflammatory

Hypertrophic cardiomyopathy has a thick _____________ that is too close to the anterior mitral leaflet, which causes outflow obstruction.

interventricular septum

The ductus arteriosus should be (kept open/closed immediately) in cases of Tetralogy of Fallot.

kept open

In patients with ventricular septal defect, the ECG most commonly demonstrates (left/right) ventricular hypertrophy.

left

The murmur of ventricular septal defect is loudest at the ____________________.

left lower sternal border

There will be hypotension and weak pulses in the (upper/lower) extremities in coarctation of the aorta.

lower

The intial treatment of hypertrophic cardiomyopathy is β-blockers (metoprolol) and/or rate-limiting Ca channel blockers (usually verapamil) to decrease _________________ and slow the heart rate and thus prolong diastolic filling and decrease outflow obstruction

myocardial contractility

A(n) (open/closed) ductus arteriosus is necessary for life in patients with hypoplastic left heart syndrome.

open

In acute rheumatic fever _____________ 0.25 to 1 mg/kg po bid (or 0.125 to 0.5 mg/kg po qid) up to 60 mg/day is recommended for inflammation of joints.

prednisone

Infantile coarctation of the aorta is located (distal/proximal) to the ductus arteriosus.

proximal

A chest x-ray for a VSD might be normal or show enlarged _____________ arteries and increased pulmonary vasculature with large shunts.

pulmonary arteries

Patent ductus arteriosus is defined as the failure of the ductus arteriosus to close, allowing communication between the aorta and the _________________.

pulmonary artery

A 2-year-old boy comes to the pediatrician with this mother because of concerns that *he occasionally "turns blue," especially when he plays* with his older brother. She describes episodes in which the boy would be playing, and within a few minutes, he begins to *develop shortness of breath* and his *lips become blueish*; both of which are *relieved upon squatting*. He is otherwise healthy and has achieved all developmental milestones. A *systolic ejection murmur* is heard on auscultation at the *left upper sternal border*. An echocardiogram is ordered which shows multiple lesions consistent with a congenital cardiac defect. Which of the following findings is the *most likely primary determinant of the severity of symptoms?*

pulmonary stenosis

The Jones criteria are used to diagnose (disease) acute ___________________.

rheumatic fever

Hypertrophic cardiomyopathy can cause _________ during exercise and may lead to sudden death in young athletes due to ventricular arrhythmia.

syncope

A crescendo-decrescendo (systolic/diastolic) murmur is heard in hypertrophic cardiomyopathy.

systolic

Ventricular septal defect causes a (systolic/diastolic) murmur.

systolic murmur

Atrial septal defects can cause both a systolic and diastolic murmur; the diastolic murmur arises from increased flow across the ____________ valve.

tricuspid valve

Tricuspid Atresia repair

3 staged repair -Blalock Taussig: shunt > Glenn > Fontan 1) Blalock Taussig Shunt at 2-3 weeks 2) Glenn shunt at 4-6 months 3) Fontan procedure at 2-3 years. (Glenn shunt is left in place)

Central cyanosis seen when absolute level of deoxygenated blood reaches:

3-5 grams/100cc blood

Which of the following is not a major criterion for diagnosing acute rheumatic fever? A. Polyarthralgia B. Sydenham's Chorea C. Migratory polyarthritis D. Erythema marginatum

A (Polyarthralgia) (Polyarthralgia is a minor criterion. All others are major criteria)

Normal O2 saturation is:

> 94%

A 3-month-old infant is brought to her pediatrician because she coughs and seems to have difficulty breathing while feeding. In addition, she seems to have less energy compared to other babies and appears listless throughout the day. She was born by cesarean section to a G1P1 woman with no prior medical history and had a normal APGAR score at birth. Her parents say that she has never been observed to turn blue. Physical exam reveals a high-pitched holosystolic murmur that is best heard at the lower left sternal border. The most likely cause of this patient's symptoms is associated with which of the following abnormalities? A. 22q11 deletion B. Deletion of genes on chromosome 7 C. Lithium exposure in utero D. Maternal alcohol consumption E. Retinoic acid exposure in utero

A (22q11 deletion) (This infant with shortness of breath, fatigue, and a holosystolic murmur best heard in the lower left sternal border most likely has a ventricular septal defect, which can be associated with 22q11 deletion syndrome (DiGeorge syndrome).

A 3-hour-old female infant becomes cyanotic and tachypneic after her first feeding. Her temperature is 37°C (98.6°F), pulse is 110/min, respirations are 65/min, and blood pressure is 80/50 mm Hg. Physical examination shows central cyanosis, but is otherwise normal. Pulse oximetry on room air shows an oxygen saturation of 80% on room air. Arterial blood gas analysis on room air shows PO2 of 30 mm Hg. Her airway is stable, and an echocardiogram was obtained and is shown below. Which of the following is the most appropriate first step in the management of this patient's condition? A. Administration of intravenous prostaglandin E1 B. Arterial switch procedure C. Dobutamine and empiric antibiotic therapy D. Emergent cardiac catheterization and atrial septostomy E. Observation pending resolution of hypoxemia

A (Administration of intravenous prostaglandin E1) (Intravenous prostaglandin E1 is the first step in the management of a patient with transposition of the great vessels, followed by surgical intervention once the patient is stabilized) (The newborn most likely has transposition of great vessels (TGV). TGV is one of the most common cyanotic congenital heart lesions in neonates. It is defined as the abnormal spatial arrangement of the great vessels, which are the aorta, pulmonary artery, superior and inferior vena cavae. A common subset is the transposition of the great arteries (TGA), in which the aorta arises out of the right ventricle and the pulmonary artery arises out of the left. Cyanosis classically presents soon after birth and progresses due to the closure of the ductus arteriosus within the first few hours of life. Once suspected, diagnosis is confirmed by imaging and laboratory studies. Initial therapy with prostaglandin E1 is a temporizing measure that maintains patency of the ductus arteriosus to enable the flow of oxygenated blood from the pulmonary artery to the aorta. Surgical treatment to correct the defect is indicated within the first 2 weeks of life, depending on the patient's stability)

A 3-year-old boy presents to his cardiologist for routine follow-up for his congenital heart disease. He was diagnosed in infancy when he presented with bluish skin shortly after birth. During the current visit, he does not appear to be in distress. A harsh systolic ejection murmur is heard over the left mid-sternal border upon auscultation, and no S2 split can be appreciated. There is no jugular vein distention, and his liver is not enlarged. His parents say that he has episodes of weakness and paleness several time a day, during which squatting improves his symptoms. A chest X-ray reveals a boot-shaped heart from right ventricular hypertrophy. Which of the following embryological malformation is most likely responsible for his condition? A. Anterosuperior displacement of the infundibular septum B. Aorticopulmonary septum spiral defect C. Ebstein anomaly D. Patent foramen ovale E. Pulmonary venous plexus anomaly

A (Anterosuperior displacement of the infundibular septum) (Tetralogy of Fallot is a right-to-left congenital heart disease with the radiological finding of a boot-shaped heart on chest x-ray due to right ventricular hypertrophy. The tetralogy arises due to anteriorsuperior displacement of the infundibular septum during embryological development) (This patient displays typical signs and symptoms of tetralogy of Fallot, a congenital right-to-left shunt caused by anterosuperior displacement of the infundibular septum. The tetralogy has the following components: Pulmonary infundibular stenosis, ventricular septal defect, overriding aorta, and right ventricular hypertrophy. The right ventricular hypertrophy causes a "boot-shaped" heart on chest X-ray, which is seen in the image. Children present with "tet" spells of cyanosis and syncope that are alleviated by squatting, which increases systemic vascular resistance, thereby temporarily reversing the right-to-left shunt. The pulmonary component of S2 is not audible. A crescendo-decrescendo systolic ejection murmur results from the right ventricular outflow obstruction, and depends on the degree of right ventricular outflow obstruction)

A 48-hour-old female infant delivered at 39 weeks' gestation has cyanosis which has progressively worsened since birth with no apparent respiratory distress. On auscultation, a clear single second heart sound is heard with no thrills or rubs, and no wheezing, ronchi, or rales. Chest radiograph is obtained which shows normal heart size and normal pulmonary vasculature. An echocardiogram, which is obtained and is shown below, shows the aorta rising anteriorly from the right ventricle. Which of the following is the most appropriate treatment option for this patient? A. Arterial switch procedure B. Fontan procedure C. Indomethacin infusion D. Intravenous prostaglandin with digitalis E. Observation

A (Arterial switch procedure) (Arterial switch procedure is indicated for a newborn with transposition of the great vessels. It needs to be performed within two weeks of life. Main explanation Transposition of the great vessels (TGV) is a congenital heart defect characterized by an abnormal spatial arrangement of any of the great vessels, including the aorta, pulmonary artery, superior and inferior vena cavae. A specific subset affecting the arteries only shows the aorta arising from the right ventricle and the pulmonary trunk arising from the left. TGV typically presents as early cyanosis, normal sized heart especially on the first few days of life (or the classic egg on string radiographic pattern), normal or increased pulmonary vascular markings and an electrocardiogram with right axis deviation. The ductus arteriosus typically closes in the first few hours of life, which explains the patient's worsening cyanosis as it no longer can correct for the transposition of great vessels. Typically the best procedure is the arterial switch procedure (Jatene procedure), which is performed in the first two weeks of life, as the left ventricle will still be able to support the systemic circulation. The initial maintenance is prostaglandin E1 to sustain patency of the ductus arteriosus and until the surgical procedure is able to be performed. This is unlikely to treat the patient's underlying problem and is only used for maintenance)

A 2-week-old infant is brought to your office by his mother for a checkup and establishment of care. They have just moved into the area, and you have not seen the baby before. The mother states that the baby was born at term and she had a normal pregnancy. On examination, the infant has a grade 3/6 harsh pansystolic heart murmur heard along the lower left sternal edge. There is no radiation of the murmur. The heart rate is 82 beats/minute and regular. There is no thrill. The blood pressure is 80/60 mm Hg. No other abnormalities are found on examination. The infant is in the 50th percentile for height and weight. What is the most likely cardiac diagnosis in this infant? A. ventricular septal defect (VSD) B. innocent cardiac murmur C. tetralogy of Fallot D. pulmonary atresia E. coarctation of the aorta

A (Ventricular septal defect (VSD)) (This infant most likely has a small VSD. Most commonly, the murmur is detected at 2 to 6 weeks of age when the infant returns for the initial checkup after hospital discharge. The typical heart murmur associated with a VSD is harsh, pansystolic, and best heard at the lower left sternal edge. Even as the VSD becomes smaller, it maintains its regurgitant characteristic of starting off with the first heart sound (holosystolic timing). As a VSD closes and becomes smaller, the murmur can actually become louder until the VSD is closed. Infants with small VSDs are well developed and acyanotic. Infants with large VSDs may have poor weight gain or show signs of congestive heart failure before 2 or 3 months of age)

An 18-year-old man comes to his primary care physician's office for a required physical examination prior to starting college. The patient says that he has been generally healthy, although he has recently noticed that he experiences shortness of breath very quickly during his daily morning runs. Physical examination shows a wide, fixed S2 splitting as well as a grade 2/6 systolic murmur in the left third intercostal space. Which of the following congenital defects is the most likely cause of the patient's symptoms? A. Atrial septal defect B. Bicuspid aortic valve C. Patent ductus arteriosus D. Tetralogy of Fallot E. Transposition of the great vessels

A (Atrial septal defect) (Atrial septal defect (ASD) is characterized by a wide fixed splitting of S2 and a systolic murmur at the left third intercostal space) (Atrial septal defect (ASD) is one of the most common congenital cardiac anomalies manifesting in adulthood. It is characterized by a defect of the interatrial septum allowing for the mixing of oxygenated and deoxygenated blood. Patients with ASD are usually asymptomatic; however, large defects can cause a left-to-right shunt, and some symptoms include easy fatigability, recurrent respiratory infections, and exertional dyspnea. The increased blood flow in the right side of the heart results in fixed wide splitting of S2 (due to increased duration of flow through the pulmonic valve, causing the valve to close later) and a systolic murmur in the third left intercostal space (because the increased flow over the valve is turbulent). If ASD is suspected, transthoracic 2-dimensional echocardiography can confirm the diagnosis, and intervention is typically surgical correction of the defect)

A 19-year-old Caucasian male collapsed from sudden cardiac arrest while playing in a college basketball game. Attempts at resuscitation were unsuccessful. Post-mortem pathologic and histologic examination found asymmetric left ventricular hypertrophy and myocardial disarray. Assuming this was an inherited condition, the relevant gene most likely affects which of the following structures? A. Cardiac cell sarcomere proteins B. Membrane potassium channel proteins C. Ryanodine receptors D. Autoimmune beta-cell antibodies E. Membrane sodium channels

A (Cardiac cell sarcomere proteins) (This patient's sudden cardiac arrest at a young age is suggestive of hypertrophic cardiomyopathy, an inherited autosomal dominant mutation of genes that encode for cardiac cell sarcomere proteins)

A 9-year-old boy is brought to the clinic by his parents for an annual wellness examination. He is a relatively healthy boy who was born at term via vaginal delivery. He is meeting his developmental milestones and growth curves and is up-to-date on his immunizations. The father complains that he is picky with his food and would rather eat pizza. The patient denies any trouble at school, fevers, pain, or other concerns. A physical examination demonstrates a healthy boy with a grade 3 midsystolic ejection murmur at the second intercostal space that does not disappear when he sits up. What is the most likely explanation for this patient's findings? A. Defect of the septum secundum B. Failure of the septum primum to fuse with the endocardial cushions C. Inflammation of the visceral and parietal pericardium D. Physiologic conditions outside the heart E. Prolonged patency of the ductus arteriosus

A (Defect of the septum secundum) (This patient most likely has an atrial septal defect (e.g., asymptomatic with grade 3 midsystolic ejection murmur). The most common and therefore most likely cause of the atrial septal defect is via a defect of the septum secundum.

A 7-year-old boy is being evaluated in pediatric cardiology clinic. He appears grossly normal, but suddenly becomes tachypneic and cyanotic when his mom takes a toy away from him. These symptoms resolve somewhat when he drops into a squatting position. Transthoracic echocardiography reveals pulmonic stenosis, a ventricular septal defect, right ventricular hypertrophy, and an overriding aorta. Which of the following best predicts the degree of cyanosis and other hypoxemic symptoms in this patient? A. Degree of pulmonic stenosis B. Size of ventricular septal defect (VSD) C. Degree of right ventricular hypertrophy (RVH) D. Degree to which aorta overrides right ventricle E. Presence of S3

A (Degree of pulmonic stenosis) (This patient's echocardiogram reveals the classic findings of tetralogy of Fallot (TOF): pulmonic stenosis, VSD, RVH, and overriding aorta. The degree of pulmonic stenosis is the best predictor of the degree of cyanosis in patients with TOF)

A 23-hour-old infant delivered vaginally at 38 weeks gestation was born with progressive cyanosis in absence of respiratory distress. Physical examination shows central cyanosis, but is otherwise normal. The patient's delivery was smooth without any complications. Subcostal echocardiogram shows failure of the aorticopulmonary septum to spiral. Which of the following conditions is most likely seen in the newborn's mother and is associated with this defect? A. Diabetes mellitus B. Epilepsy C. Hypertension D. Turner's syndrome E. Type I bipolar disorder

A (Diabetes mellitus) (Diabetes mellitus is a maternal condition which has been associated with fetal congenital heart defects including transposition of the great vessels) (The newborn most likely has transposition of great vessels (TGV). TGV is one of the most common cyanotic congenital heart lesions in neonates. It is defined as the abnormal spatial arrangement of the great vessels, which are the aorta, pulmonary artery, superior and inferior vena cavae. A common subset is transposition of the great arteries, in which the aorta arises out of the right ventricle and the pulmonary artery arises out of the left. Cyanosis classically presents soon after birth and progresses due to closure of the ductus arteriosus within the first few hours of life. Inheritance of the defect is multifactorial, although there is a high association of TGV in infants with diabetic mothers. Once suspected, diagnosis is confirmed by imaging and laboratory studies. Surgical treatment to correct the defect is indicated within the first 2 weeks of life, depending on the patient's stability)

An 8-year-old African-American male is found to have a holosystolic, harsh-sounding murmur upon physical examination. The murmur is best appreciated at the left sternal border, and is found to be louder when the patient squats. Which of the following is the most likely diagnosis? A. Ventricular septal defect B. Patent ductus arteriosus C. Atrial septal defect D. Tricuspid atresia E. Aortic stenosis

A (Ventricular septal defect) (The murmur of a ventricular septal defect (VSD) is holosystolic and best heard over the "tricuspid area", or the lower left sternal border, with radiation to the right lower sternal border because this is the area which overlies the defect) (A VSD is a connection between the left and right ventricles. Blood flows from the high pressure left ventricle to the lower pressure right ventricle during systole, producing a murmur. During a squatting (or hand grip) maneuver, total peripheral resistance is increased, forcing more blood through the defect and causing a louder murmur)

A 1-week-old infant boy comes to the office for his first visit. Antenatal and birth history show no complications. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. He is a well-appearing neonate. There is *no cyanosis* and capillary refill is <2 seconds. On auscultation, a *III/VI harsh holosystolic murmur* is heard at the *left of the sternum with radiation to the back.* Which of the following is the most appropriate next step in management? A. Echocardiogram B. Electrocardiogram C. Emergent surgical repair D. Indomethacin E. Prostaglandin E1

A (Echocardiogram) (Ventricular septal defects can be heard best at the left lower sternal edge, as harsh, holosystolic murmurs. When one is suspected clinically, an echocardiogram is required as the results will guide treatment) (This patient has a ventricular septal defect (VSD). Two-dimensional echocardiography, with Doppler echocardiography and color flow imaging, can be used to determine the size and location of virtually all VSDs. Doppler echocardiography provides additional physiologic information (eg: right ventricle (RV) pressure, pulmonary artery (PA) pressure, and interventricular pressure difference). (The sound heard on auscultation is produced by left-to-right shunting through the defect. Harsh murmurs are actually more likely to indicate a smaller VSD rather than a larger one, because the blood going through a small VSD experiences greater resistance than blood going through a large one. Some small VSDs will self-resolve as the ventricular septum grows and thickens with age. If a VSD persists, however, the right heart and pulmonary circuit are continually exposed to higher than normal pressures. This could lead to pulmonary hypertension and a reversal of the shunt from left-to-right to right-to-left (this is known as Eisenmenger's syndrome). In this case, the child would become cyanotic as deoxygenated blood is shunted into the systemic circulation) (Medical treatment of VSDs involves regular follow-up with a cardiologist as well as supplemental nutrition (for VSDs causing failure to thrive), blood pressure control (with diuretics or ACE inhibitors), contractility improvement (digoxin), and surgical repair) (Indomethacin can be used to close a symptomatic patent ductus arteriosus, by reducing the production of prostaglandin E1. A patent ductus arteriosus causes a constant "machine-like" murmur)

A 10-year-old girl comes to the pediatric clinic because of pain in her right wrist preventing her from writing in school. Her mother mentions that two days ago, she had elbow pain which they believed was from too much basketball because last week after a basketball game she complained of left knee pain for a few days. She has been otherwise healthy except for a bad sore throat about a month ago. On physical examination she has a fever of 100.6F and a II/VI systolic murmur best heard at the apex. While labs are pending, an electrocardiogram is ordered, which reveals a prolonged PR interval. Which of the following other findings might be expected in this patient? A. Elevated erythrocyte sedimentation rate B. Retinal hemorrhages C. Metabolic acidosis D. Positive anti-nuclear antibodies E. Rash on cheeks and bridge of nose

A (Elevated erythrocyte sedimentation rate) (This patient meets the Jones criteria for acute rheumatic fever, most common in children aged 5-15 years old and 2-4 weeks after a pharyngeal infection by group A streptococcus. Jones criteria requires group A streptococcus infection followed by 2 major or 1 major and 2 minor manifestations. (This patient meets the Jones criteria for acute rheumatic fever, most common in children aged 5-15 years old and 2-4 weeks after a pharyngeal infection by group A streptococcus. Jones criteria requires group A streptococcus infection followed by 2 major or 1 major and 2 minor manifestations, listed below) (The five major manifestations spell JONES (pretend the heart is shaped like an O): 1. Joints: migratory arthritis, predominantly large joints 2. Carditis and valvulitis 3. Nodules (subcutaneous) 4. Erythema marginatum 5. Sydenham chorea (central nervous system involvement) The four minor manifestations are: 1. Arthralgia 2. Fever 3. Elevated acute phase reactants (erythrocyte sedimentation rate, C-reactive protein) Prolonged PR interval (Pathophysiology is antibody cross-reactivity between group A streptococcus's M protein and antigens in myocardium, joints, and central nervous system tissue. This molecular mimicry causes a Type II hypersensitivity reaction. Rheumatic heart disease can be temporary or chronic. It can predispose patients to left heart dilation, left ventricular hypertrophy, congestive heart failure, arrhythmia, and bacterial endocarditis) (Acute treatment is with anti-inflammatories such as corticosteroids. Patients should start antibiotics to eradicate group A streptococcus regardless of whether pharyngitis is present. Household contacts should receive throat cultures)

Which of the following physical findings is suggestive of atrial septal defect? A. Fixed split S2 B. Increased pulse pressure C. Continuous mechanical murmur D. Difference in blood pressure between the left and right arm

A (Fixed split S2)

A 23-year-old male presents with syncope. On physical examination you note a medium-pitched, mid-systolic murmur that decreases with squatting and increases with straining. Which of the following is the most likely diagnosis? A. Hypertrophic cardiomyopathy B. Aortic stenosis C. Mitral regurgitation D. Pulmonic stenosis

A (Hypertrophic cardiomyopathy)

13-year-old girl is brought to the clinic by her parents for joint pain for the past 4 days. She reports a 6/10, intermittent, dull pain in her knees and ankles. The pain does not stay in one place and over the past day, her right knee has become red and swollen. She endorses subjective fever and fatigue but denies chest pain, dizziness, abdominal pain, gastrointestinal signs, or urinary changes. Her past medical history is unremarkable except for an episode of sore throat 3 weeks ago. A physical examination demonstrates an erythematous, swollen right knee that is tender upon palpation and annular red macules on the upper left arm.. What finding would you expect in this patient? A. Increased anti-streptolysin O titers B. Positive anti-cyclic citrullinated peptide antibodies C. Positive anti-dsDNA antibodies D. Presence of needle-shaped crystals under polarized light E. Purulent synovial fluid with WBC > 50,000/mm^3

A (Increased anti-streptolysin O titers) (This patient likely has rheumatic fever as demonstrated by her migratory joint pain, erythema marginatum, and fever in the setting of recent Streptococcus pyogenes infection (e.g., sore throat). Patients with recent S. pyogenes infection will have increased anti-streptolysin O titers)

A 12-year-old girl with an autosomal dominant mutation in myosin-binding protein C is being evaluated by a pediatric cardiologist. The family history reveals that the patient's father died suddenly at age 33 while running a half-marathon. What was the likely finding on histological evaluation of her father's heart at autopsy? A. Myocyte disarray B. Amyloid deposits C. Eosinophilic infiltration D. Wavy myocytes E. Viral particles

A (Myocyte disarray) (Based on the patient's mutation and family history, the patient described above likely has Hypertrophic Cardiomyopathy (HCM). About half of HCM cases are due to an autosomal dominant genetic disease that, along with myocyte disarray, has the histological hallmarks of fibrosis and myocyte hypertrophy) (Several autosomal gene mutations in sarcomere proteins may lead to HCM, including myosin-binding protein C, beta-myosin heavy chain, and the cardiac troponins. These mutations result in a decrease in the effectiveness of cardiac contraction, thereby increasing the amount of work the heart must perform. This leads to the enlargement of the heart and the histological manifestations described above (myocyte proliferation, hypertrophy, and disarray)

A 17-year-old male collapses 25 minutes into a soccer game. He is unresponsive and pulseless. Despite adequate resuscitation by a bystander, the patient is pronounced dead when the ambulance arrives. The patient had no past medical history other than a heart murmur as a child and he took no medications. His family history is notable for an uncle who died suddenly of unknown causes at the age of 25. A mutation in which of the following proteins most likely contributed to this patient's condition? A. Myosin heavy chain B. Dystrophin C. Tropomyosin D. Fibrilin E. Elastin

A (Myosin heavy chain) (The most likely diagnosis in this patient is hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy occurs due to mutations in sarcomere proteins such as myosin heavy chain) (The clinical presentation of sudden cardiac death during exertion in an otherwise healthy individual suggests a diagnosis of hypertrophic cardiomyopathy. This diagnosis is supported by the finding of a childhood murmur and a family history of sudden death. Hypertrophic cardiomyopathy is caused by autosomal dominant mutations in various sarcomere proteins such as myosin heavy chain and troponin. Structurally, this mutation leads to an asymmetrically thickened left ventricular wall and enlarged septum. Disordered myofibrils will be seen on histologic examination of hearts in patients with this condition. Clinically, hypertrophic cardiomyopathy presents as sudden death in young athletes due to either fatal arrhythmias or severe left ventricular outflow tract obstruction)

What preventative health practices should be followed for the child with the asymptomatic VSD murmur described here? A. prophylaxis against bacterial endocarditis if dental work is to be done B. cardiac catheterizations every 3 months until resolution C. echocardiograms every 3 months until resolution

A (prophylaxis against bacterial endocarditis if dental work is to be done) (The only prophylaxis that needs to be followed in this child is protection with antibiotic therapy (penicillin, amoxicillin, or erythromycin for patients allergic to penicillin) before any dental procedure or any other procedure that would increase the probability of bacterial endocarditis. This obviously can be discontinued when the defect closes. Serial echocardiograms are indicated. Different cardiologists have different recommendations for the frequency of examination, but every 6 to 12 months is the range, not every 3 months. Cardiac catheterization is not indicated for this lesion)

Where are murmurs for ASD best heard? A. second intercostal left sternal edge B. at the upper left sternal border and in the infraclavicular area C. the apex with radiation to the left axilla D. the second and third left intercostal space with radiation to the left shoulder

A (second intercostal left sternal edge)

A 16-year-old male presents with complaint of syncope after basketball practice today. Physical examination reveals a systolic murmur along the left sternal border that increases with Valsalva maneuver. An electrocardiogram reveals left ventricular hypertrophy. Echocardiogram shows asymmetric left ventricular hypertrophy with a hypercontractile left ventricle. Which of the following is the initial medication of choice in this patient? A. Metoprolol (Lopressor) B. Cozaar C. Lisinopril (Zestril) D. Hydrochlorothiazide (Diuril)

A. (Metoprolol (Lopressor)) (Beta-blockers are the initial drug of choice in a symptomatic patient with hypertrophic cardiomyopathy. Avoid nitrates and other drugs that decrease preload (eg, diuretics, ACE inhibitors, angiotensin II receptor blockers) because these decrease LV size and worsen LV function)

____________________ are the antibody titers that are characteristically elevated in rheumatic fever.

Anti-streptolysin O

A 5-year-old girl comes to the pediatrician's office for a physical examination prior to starting school. Her mother says that she "does very well at home" and has reached all developmental milestones. The child is currently sitting in the room and is playing comfortably. Physical examination shows a *systolic murmur at the left third intercostal space* accompanied by a *loud first sound* and a *split second heart sound* that shows no variance with respiration. Which of the following congenital defects is the most likely cause of these findings?

Atrial Septal Defect

A healthy 7-year-old girl who has reached all developmental milestones. On examination, the precordium is hyperdynamic with a prominent right ventricular heave. A grade III/VI systolic ejection murmur is present in the 2nd left intercostal space (pulmonic position) with an early to mid-systolic rumble and a fixed splitting of the second heard sound (s2) during inspiration and expiration. What is the most likely diagnosis?

Atrial Septal Defect

An 16-year-old boy comes to his primary care physician's office for a required physical examination prior to starting college. The patient says that he has been generally healthy, although he has recently noticed that &he experiences *shortness of breath during his daily morning runs.* Physical examination shows a *wide, fixed S2 splitting* as well as a *grade 2/6 systolic murmur in the left third intercostal space.* Which of the following congenital defects is the most likely cause of the patient's symptoms?

Atrial Septal Defect

What defect? Left to right shunting for years Increased pulmonary blood flow If pulmonary hypertension develops, then reversal with right to left shunting occurs Fixed split S2 and pulmonary flow murmur.

Atrial Septal Defect

Absence of a tricuspid valve requires an ______________ defect for viability.

Atrial and ventricular septal defect

What defects are associated with Ebstein's anomaly?

Atrial septal defect Ventricular septal defect Patent foramen ovale Patent ductus arteriosus Mitral valve prolapse Bicuspid AV Accessory pathways. Variable right-o-left shunting with cyanosis.

Syndrome and heart defect: Trisomy 21

Atrioventricular septal defect (AVSD) Ventricular septal defect (VSD) Atrial septal defect (ASD) Patent ductus arteriosus (PDA)

A 12 month-old child with tetralogy of Fallot is most likely to have which of the following clinical features? A. Chest pain B. Cyanosis C. Convulsions D. Palpitations

B (Cyanosis)

Which of the following medications should be avoided in patients with hypertrophic obstructive cardiomyopathy? A. Aspirin B. Digoxin C. Disopyramide D. Acetaminophen E. Atenolol

B (Digoxin) (Hypertrophic obstructive cardiomyopathy is an autosomal-dominant transmitted disorder that is characterized by an enlarged cardiac septum, which obstructs blood flow from the left ventricle. Symptoms include dizziness, light-headedness, palpitations, chest pain, dyspnea, or syncope with physical exertion. The most important complication of the disease is sudden death. The condition has an annual incidence of 4% to 6% in children, and 2% of adults are affected. Signs include pulses that are bifid and brisk in upstroke and a systolic-ejection-type murmur positioned along the left sternal border, which becomes louder with movements that decrease venous return (afterload), such as standing or Valsalva's maneuver. Movements that increase venous return (afterload), such as squatting, reduce the murmur. ECG usually shows evidence of LVH and septal Q waves in the lateral leads. Diagnosis is accomplished by echocardiogram. First-line treatment is accomplished with the use of β-blockers. Disopyramide (Norpace) may be used as an alternative. Verapamil has also been used frequently on an empiric basis especially in atrial fibrillation but its effect is unpredictable, and acute hemodynamic collapse is described in patients with substantial gradients or severe diastolic dysfunction)

A 6-day-old boy is brought to the emergency room by his parents for difficulty breathing. He was delivered at home via vaginal birth with no complications. The mom received minimal prenatal care as she wanted the most natural process for her child. The mom reports that he has been relatively healthy expect for when he feeds he gets a little fussy. However, these episodes self-resolve after he curls up his legs. A physical examination demonstrates a baby in acute distress with subcostal retractions and cyanosis at the distal extremities. A 5/6 systolic ejection murmur is heard at the left upper sternal border. What is the best next step in terms of managing this patient? A. Administer warming blankets B. Give prostaglandin E1 C. Give antibiotics D. Immediate surgical repair E. Obtain lumbar puncture for CSF analysis

B (Give prostaglandin E1) (This patient is suffering from severe cyanosis secondary to tetralogy of Fallot (e.g., recurrent tet spells, cyanosis, and loud ejection murmur). The most appropriate method of management is to administer intravenous prostaglandin E1 therapy to maintain ductal patency and pulmonary flow pending surgical repair)

An newborn infant comes to the attention of the neonatal care unit because he started having heavy and rapid breathing. In addition, he was found to be very irritable with pale skin and profuse sweating. Finally, he was found to have cold feet with diminished lower extremity pulses. Cardiac auscultation reveals a harsh systolic murmur along the left sternal border. Notably, the patient is not observed to have cyanosis. Which of the following treatments would most likely be effective for this patient's condition? A. Leukotriene E4 B. Prostaglandin E1 C. Prostaglandin E2 D. Prostaglandin I2 E. Thromboxane A2

B (Prostaglandin E1) (This non-cyanotic patient with cold feet, diminished pulses, and a harsh systolic murmur most likely has coarctation of the aorta, which can be treated with prostaglandin E1 to maintain a patent ductus arteriosus) (Coarctation of the aorta is a narrowing of the aorta that usually occurs proximal to the ductus arteriosus in infants. Since infants are not able to communicate their symptoms, common findings that should be noted are irritability, pale skin, profuse sweating, cold feet, and diminished pulses. Cardiac auscultation will reveal a harsh systolic murmur along the left sternal border where blood flows past the obstruction. Infants with this disorder should be given prostaglandin E1 to maintain patency of the ductus arteriosus prior to surgical repair)

A 4-month-old is noted to have a grade 3/6, harsh, systolic ejection murmur heard at the left upper sternal border. The mother reports that the child's lips occasionally turn blue during feeding. A cardiologist recommends surgery. Later, the physician remarks that the infant's congenital abnormality was related to a failure of neural crest cell migration. Prior to surgery, which of the following was a likely finding? A. Atrial septal defect B. Pulmonic stenosis C. Triscuspid atresia D. Coarctation of the aorta E. Transposition of the great vessels

B (Pulmonic stenosis) (The presentation of cyanotic spells with activity (feeding) are most consistent with Tetralogy of Fallot (TOF), a congenital cardiac defect characterized by (1) pulmonic stenosis, (2) ventricular septal defect, (3) overriding aorta, and (4) right ventricular hypertrophy)

Which of the following is associated with acute rheumatic fever A. Manning criteria B. Revised Jones criteria C. Ranson criteria D. Duke criteria

B (Revised Jones criteria) (Revised Jones criteria is used to make a diagnosis of acute rheumatic fever. It consists of 5 major and 4 minor criteria and an absolute requirement for evidence (microbiologic or serologic) of recent Group A beta hemolytic streptococcus infection)

What is the most common pathologic murmur in childhood? A. ASD B. VSD C. patent ductus arteriosus D. Mitral valve prolapse

B (VSD) (The most common pathologic cardiac murmur in childhood is VSD. With a VSD, the cardiac murmur is often not present at birth but is first heard at the 2- to 4-week well-baby checkup. As discussed previously, the most common outcome of this congenital heart defect is spontaneous closure. In some cases, however, surgical closure is indicated. The most uncommon scenario is the development of congestive cardiac failure secondary to VSD. The relative frequencies of pathologic cardiac murmurs in childhood are as follows: VSD, 38%; ASD, 18%; pulmonary valve stenosis, 13%; pulmonary artery stenosis, 7%; aortic valve stenosis, 4%; patent ductus arteriosus, 4%; mitral valve prolapse, 4%; and all others, 11%)

A 32-year-old woman presents to the emergency department with 2 hours of left-sided weakness. Her husband reports that she had been complaining of pain and swelling in her right calf for several weeks prior to this event. The couple had recently returned from a vacation in Europe. What ausculatory finding would explain the mechanism by which her more recent complaint occurred? A. Holosystolic murmur radiating to the axilla B. Wide, fixed splitting of S2 C. Crescendo-decrescendo murmur heard loudest at the right second intercostal space D. Holosystolic murmur that increases on inspiration E. An S2 that splits during expiration

B (Wide, fixed splitting of S2) (This woman has suffered a thromboembolic stroke from a deep vein thrombus (DVT). A wide, fixed splitting of S2 is consistent with an ASD, which would allow direct communication from the venous system to the arterial system without traversing the lungs, thereby causing her paradoxical embolism)

What diagnostic/ therapeutic procedure? Invasive evaluation of pressure and blood flow Evolved to *primarily therapeutic modality* -Close holes -Open valves, narrowings

Cardiac Catheterization

You are asked to evaluate a term infant in the delivery room. He was born a few minutes earlier by spontaneous vaginal delivery with Apgar scores of 9 and 9. The mother had good prenatal care and a normal pregnancy. No significant family history is noted. On physical examination, the infant appears alert and is active and crying. Acrocyanosis is noted on extremities. A grade 2/6 soft, systolic murmur is audible at the left upper sternal border. Otherwise, the examination is completely normal. What should be your next step in the management of this patient? A. order an echocardiogram B. perform a second detailed physical exam at 24 hours C. order an ECG and chest radiograph D. get a cardiology consultation

B (perform a second detailed physical exam at 24 hours) (This is the transient systolic murmur of patent ductus arteriosus. It is audible at the upper left sternal border and in the left infraclavicular area on the first day, and it usually disappears soon thereafter. As in older infants and children, not all heart murmurs in neonates are pathologic. More than 50% of term-born infants are found to have innocent systolic murmurs at some time during the first week of life. The prevalence is even higher in premature infants. The incidence of structural congenital heart disease is estimated to be less than 1% of all live births. The infant should have a detailed examination at 24 hours and again before discharge before any further decisions are made. Acrocyanosis, a normal phenomenon, should also be distinguished from central cyanosis)

Which of the following medications is indicated in the treatment of patent ductus arteriosus? A. prednisolone B. dexamethasone C. indomethacin D. cyanoacrylate tissue adhesive

C (indomethacin) (Give premature infants a prostaglandin inhibitor (eg, indomethacin) to close the PDA; surgical closure has not been shown to improve outcomes. Prostaglandin inhibitors are usually ineffective in full-term infants, but catheter-delivered occlusion devices or surgery is typically beneficial)

Circulation inside the fetal heart

Blood enters the right atrium, the chamber on the upper right side of the heart. When the blood enters the right atrium, most of it flows through the foramen ovale into the left atrium. Blood then passes into the left ventricle (lower chamber of the heart) and then to the aorta, (the large artery coming from the heart). From the aorta, blood is sent to the heart muscle itself in addition to the brain. After circulating there, the blood returns to the right atrium of the heart through the superior vena cava. About two thirds of the blood will pass through the foramen ovale as described above, but the remaining one third will pass into the right ventricle, toward the lungs. In the fetus, the placenta does the work of breathing instead of the lungs. As a result, only a small amount of the blood continues on to the lungs. Most of this blood is bypassed or shunted away from the lungs through the ductus arteriosus to the aorta. Most of the circulation to the lower body is supplied by blood passing through the ductus arteriosus. This blood then enters the umbilical arteries and flows into the placenta. In the placenta, carbon dioxide and waste products are released into the mother's circulatory system, and oxygen and nutrients from the mother's blood are released into the fetus' blood.

A 5-year-old boy comes to the office because of a routine examination. He is up to date on his immunizations, but has received little other medical treatment during his life. His mother notes that when he was born, she was told that he had a small "hole inside of his heart." She is not aware of any other health issues, although she does note that *he seems to get tired more easily as of late.* Examination shows the boy is afebrile. The *point of maximum impulse is displaced leftward.* There is no clubbing or edema visible. Cardiac auscultation shows a I/VI *holosystolic murmur at the lower left sternal border.* The lungs are clear to auscultation. Which of the following is most likely to be observed on chest X-ray? A. Bibasilar pulmonary infiltrates B. Blunted costovertebral angles C. Cardiomegaly D. Dilated aortic root E. Left-shifted trachea

C (Cardiomegaly) (Ventricular septal defect is characterized by worsening fatigue, a holosystolic murmur, and a displaced point of maximal impulse. Both left and right ventricles are likely to be enlarged in a patient like this, who has had little medical care) (This child is likely experiencing heart failure as a result of a ventricular septal defect. His history given by the mother is confirmed by the examination finding of a holosystolic murmur and displaced PMI. PMI displacement has a 95% specificity for detecting cardiac dilatation secondary to heart failure) (The subtle nature of the murmur (grade I/VI) likely indicates that the defect is probably rather large; smaller defects tend to produce louder murmurs because they have higher flow resistance and thus create more turbulent flow) (Cardiomegaly is likely to be present in this child as the left ventricle hypertrophies in an attempt to pump enough blood to the rest of the body. The right ventricle may also dilate as a result of the extra blood volume it receives from the septal defect)

A 9-year-old male visited his primary care physician for a check-up three months after a throat infection. Upon examination, the patient exhibits painless subcutaneous nodules on the back of the wrist, the outside elbow, and the front of the knees, as well as inflammation in the joints of the lower extremities. Which of the following symptoms is most likely to also be present in this patient? A. Renal failure B. Hepatomegaly C. Chorea D. Pleural effusion E. Butterfly rash

C (Chorea) (The clinical picture of polyarthritis of the lower extremities and subcutaneous nodules following a pharyngitis is consistent with rheumatic fever (RF). RF may result in Sydenham's chorea, a characteristic series of rapid, purposeless movements of the face and arms) (Sydenham's chorea typically occurs 2-3 months after a group A beta-hemolytic streptococcal (GAS) throat infection and is reported to occur in 20-30% of patients with acute rheumatic fever. It is postulated that this chorea occurs as a result of an autoimmune cross-reaction between GAS antigen and cells within the basal ganglia. Additional clinical manifestations of rheumatic fever include carditis, arthritis, erythema marginatum, and subcutaneous nodules)

A 26-year-old man is brought to the emergency department because he collapsed while playing basketball 20 minutes ago. Medical history includes unexplained chest pain and shortness of breath while exercising for four years. Family history includes an uncle who died of an unknown cardiac pathology at the age of 23. Cardiac auscultation shows a 2/6 systolic murmur is heard at the left of the sternum between the first two ribs. The murmur becomes louder when the patient performs a Valsalva maneuver. Which of the following is the most likely diagnosis? A. Aortic stenosis B. Aortic regurgitation C. Hypertrophic obstructive cardiomyopathy D. Pulmonary stenosis E. Mitral valve prolapse

C (Hypertrophic obstructive cardiomyopathy) (Hypertrophic cardiomyopathy (HCM) is a primary disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is hypertrophied. Murmur due to HCM will increase in intensity with any maneuver that decreases the volume of blood in the left ventricle (such as standing abruptly or the strain phase of a Valsalva maneuver). (Hypertrophic cardiomyopathy (HCM) is a primary disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is hypertrophied (thickened) without any obvious cause, creating functional impairment of the cardiac muscle. The symptoms of HCM include dyspnea (shortness of breath) due to stiffening and decreased blood filling of the ventricles, exertional chest pain due to ischemia of the hypertrophied muscle, uncomfortable awareness of the heart beat (palpitations) due to the aforementioned ischemia, as well as disruption of the electrical system running through the abnormal heart muscle, lightheadedness, fatigue, fainting (called syncope), and sudden cardiac death. On auscultation, the heart murmur of HCM will sound similar to the murmur of aortic stenosis. However, a murmur due to HCM will increase in intensity with any maneuver that decreases the volume of blood in the left ventricle (such as standing abruptly or the strain phase of a Valsalva maneuver). This is because by decreasing left ventricular filling, the left ventricular outflow tract obstruction which occurs in HCM worsens, creating more turbulent blood flow, and a louder murmur)

A male newborn is born at 35 weeks' gestation following an uncomplicated pregnancy. Apgar scores are 9 and 9 at 1 and 5 minutes, respectively. He appears to be breathing faster than other newborns in the nursery. His pulse is 153/min. Cardiac examination shows a murmur that is heard throughout systole and diastole. Over the next 3 days, the newborn remains tachypneic, with no change in the murmur. ECG shows no congenital heart anomaly. Administration of which of the following is the most appropriate next step in management? A. Acetaminophen B. Ampicillin C. Indomethacin D. Prostaglandin E1 E. Vitamin K

C (Indomethacin) (Patent ductus arteriosus (PDA) is characterized by tachypnea and a continuous machine-like murmur on cardiac auscultation. Indomethacin, a nonsteroidal anti-inflammatory drug that inhibits prostaglandin synthesis, is the treatment of choice to promote closure of a PDA, particularly in preterm infants) (This preterm infant most likely has a patent ductus arteriosus (PDA). During gestation, the ductus arteriosus is a shunt between the pulmonary artery and the aorta that bypasses the pulmonary circulation to deliver oxygenated blood to the body. When the infant starts to breathe upon delivery, the systemic resistance becomes greater than the pulmonary resistance, promoting closure of fetal shunts (foramen ovale, ductus venosus, ductus arteriosus). If the ductus arteriosus remains open after birth, blood is shunted from the systemic to the pulmonary circulation, resulting in decreased delivery of oxygenated blood to the peripheral tissues. Consequently, infants will be characterized by tachypnea as well as a characteristic continuous machine-like murmur. Since the patient has no congenital heart defects (transposition of the great vessels, tricuspid atresia, tetralogy of Fallot), closure of the PDA is indicated. Indomethacin, a nonsteroidal anti-inflammatory drug (NSAID) that inhibits prostaglandin synthesis, is considered first-line medical therapy for a PDA)

A 2-year-old boy is brought to a pediatrician because his parents have noticed that he seems to be getting tired very easily at home. Specifically, they have noticed that he is often panting for breath after walking around the house for a few minutes and that he needs to take naps fairly often throughout the day. He has otherwise been well, and his parents do not recall any recent infections. He was born at home, and his mom did not receive any prenatal care prior to birth. Physical exam reveals a high-pitched, harsh, holosystolic murmur that is best heard at the lower left sternal border. No cyanosis is observed. Which of the following oxygen tension profiles would most likely be seen in this patient? (LV = left ventricle, RV = right ventricle, and SC = systemic circulation). A. LV: decreased, RV: increased, SC: decreased B. LV: decreased, RV: normal, SC: decreased C. LV: normal, RV: increased, SC: normal D. LV: normal, RV: normal, SC: decreased D. LV: normal, RV: normal, SC: normal

C (LV: normal, RV: increased, SC: normal) (This patient with fatigue, shortness of breath, and a holosystolic murmur most likely has a ventricular septal defect, which would present with increased oxygen tension in the right ventricle and normal oxygen tension in the other chambers (RV) as well as in the systemic circulation.

A 10-year-old boy presents to the pediatrician with a three day history of sore throat and fever of 101°F (38.3°C). On physical exam, his oral mucosa shows exudate on swollen tonsils. He also has tender anterior cervical lymph nodes. A rapid strep test returns negative. Three weeks later, the boy presents with painful joints and nodular swelling on his skin. Erythrocyte sedimentation rate (ESR) is obtained and found to be 76 mm/hr. A reaction to which of the following compounds is most likely responsible for the patient's return presentation? A. Streptolysin O B. Streptokinase C. M protein D. Protein F E. Hyaluronic acid capsule

C (M protein) (The patient likely has Streptococcus pyogenes pharyngitis with subsequent development of rheumatic fever. Rheumatic fever is caused by antibodies that develop against the M protein in Group A Streptococcus (GAS)) (As the rapid strep test is not a highly sensitive test, up to 25% of infections can be missed. This patient likely had a false negative result and therefore did not receive the antibiotic treatment necessary to prevent rheumatic fever. A diagnosis of rheumatic fever is supported by his symptoms of joint pain, subcutaneous nodules, as well as an elevated ESR. M protein is a GAS cell wall protein that is recognized by antibodies that subsequently cause the inflammatory response in rheumatic fever)

A 7-year-old girl comes to the pediatric clinic because of 3 weeks of fatigue and shortness of breath. Her mother notes that she has been fairly healthy for the duration of her life, except for a bad sore throat about 5 months ago. On examination, she appears fatigued, but is appropriately oriented and responsive, and is generally non-toxic appearing. She is afebrile. Cardiac examination shows a widely split S2, a quiet S1, and point of maximum impulse displaced to the left. Which of the following cardiac abnormalities has likely developed in this patient? A. Wolff-Parkinson-White syndrome B. Aortic aneurysm C. Mitral insufficiency D. First degree heart block E. Coronary atherosclerosis

C (Mitral insufficiency) (Rheumatic heart disease is caused by autoimmune cross-reactivity following a Streptococcal infection. Mitral valve insufficiency is the most common valvular lesion seen in this condition, but tricuspid insufficiency may develop as well. (This child is likely suffering from mitral valve insufficiency due to rheumatic heart disease. This disease is a consequence of autoimmune cross-reactivity following a throat infection with Streptococcus pyogenes. Rheumatic heart disease is the most common cause of mitral valvular diseases. Although rheumatic heart disease and acute rheumatic fever are uncommon in wealthier countries, this child has had poor medical follow-up and is therefore at similar risk. The most appropriate treatment for acute rheumatic fever and rheumatic heart disease is long-term penicillin prophylaxis, the duration of which depends upon the severity of disease)

A month-old infant is brought to the office by her parents because of concerns about *poor feeding* and *little weight gain since birth*. She was born at 35 weeks' gestation via spontaneous vaginal delivery to a healthy mother following an uncomplicated pregnancy. She appears comfortable with *no signs of cyanosis*. Her temperature is 37ºC (98.6ºF), pulse is 150/min, respirations are 45/min, and blood pressure is 105/40 mm Hg. Pulse oximetry shows an oxygen saturation of 96%. Physical examination shows a morphologically normal infant with a soft and flat anterior fontanel. Cardiac examination shows a *murmur heard throughout systole and diastole at the left second intercostal space at the sternal border* on auscultation. The extremities are well perfused with strong peripheral pulses. Which of the following is the most likely diagnosis? A. Atrial septal defect B. Eisenmenger syndrome C. Patent ductus arteriosus D. Postductal coarctation of the aorta E. Ventricular septal defect

C (Patent ductus arteriosus) (Patent ductus arteriosus can cause failure to thrive, clubbing of the lower extremities, and frequent respiratory infections but does not cause cyanosis. Physical examination findings show wide pulse pressure and a continuous machine-like murmur. The diagnosis can be confirmed by echocardiography. (The ductus arteriosus is normally patent during fetal life to allow for delivery of oxygenated blood from the placenta to the fetal systemic circulation by bypassing the pulmonary circulation. Continued patency depends on the local production of prostaglandin (PG) E2, which usually stops after birth and allows spontaneous closure of the ductus arteriosus. A patent ductus arteriosus (PDA) is defined by a failure of the ductus arteriosus to close within the first few days of life, leading to left-to-right shunting from the aorta into the pulmonary artery. Small defects are typically asymptomatic, but patients with large defects may be characterized by failure to thrive, poor weight gain, clubbing of the lower extremities (due to intermittent shunting of deoxygenated blood from the pulmonary circulation to the descending aorta), frequent respiratory infections, or (rarely) signs of right heart failure. Findings on physical examination include wide pulse pressure and a continuous machine-like murmur. The diagnosis is confirmed by echocardiography with Doppler. Treatment is with indomethacin (to inhibit PG synthesis) or surgical closure if medical therapy fails. Risk factors include prematurity, female gender, and first-trimester maternal rubella infection)

A G1P0 woman gives birth to a boy at 38 weeks' gestation. The child's height and weight are in the 10th and 12th percentiles, respectively. The mother's past medical history is notable for poorly controlled diabetes mellitus and bipolar disorder. Twenty-four hours after birth, the child is noted to have blue extremities in the newborn nursery. His temperature is 99.3°F (37.4°C), blood pressure is 90/50 mmHg, pulse is 130/min, and respirations are 28/min. He is breathing rapidly with nasal flaring and intercostal retractions. A loud S2 is heard with a continuous murmur. A chest radiograph shows an "Egg on a string" appearance of the cardiac shadow. The child is taken to the neonatal intensive care unit. Which of the following is most appropriate to administer to this patient? A. Indomethacin B. Prostacyclin C. Prostaglandin E1 D. Prostaglandin E2 E. Thromboxane A2

C (Prostaglandin E1) (The child in this vignette presents with cyanosis, tachypnea, progressive respiratory failure, and an egg-shaped heart suggestive of transposition of the great vessels (TGV). Prostaglandin E1 (PGE1) should be immediately administered in this condition) (In TGV, the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle, leading to separate parallel pulmonary and systemic circulations. An arteriovenous communication such as a patent ductus arteriosus (PDA) or ventricular septal defect (VSD) must exist to allow deoxygenated blood to enter the pulmonary circulation and become oxygenated. This mixing of arterial and venous blood is required to be compatible with life. PGE1 should be given immediately as it maintains the PDA until definitive surgical repair is possible)

A 2-year-old boy comes to the pediatrician with this mother because of concerns that he occasionally "turns blue," especially when he plays with his older brother. She describes episodes in which the boy would be playing, and within a few minutes, he begins to develop shortness of breath and his lips become blueish; both of which are relieved upon squatting. He is otherwise healthy and has achieved all developmental milestones. A systolic ejection murmur is heard on auscultation at the left upper sternal border. An echocardiogram is ordered which shows multiple lesions consistent with a congenital cardiac defect. Which of the following findings is the most likely primary determinant of the current severity of symptoms? A. Atrial septal defect B. Overriding aorta C. Pulmonary stenosis D. Right ventricular hypertrophy E. Ventricular septal defect

C (Pulmonary stenosis) (Pulmonary stenosis is the primary determinant of the severity and clinical presentation of tetraology of Fallot) (Tetraology of Fallot (TOF) is the most common cyanotic congenital heart disease. It consists of pulmonary stenosis, an overriding aorta, ventricular septal defect, and right ventricular hypertrophy. Of these four malformations, the degree of pulmonary stenosis is the primary determinant of the severity of symptoms. TOF typically presents in infancy or early childhood with shortness of breath and fatigue after exertion. By the ages of 1-2 years, children begin to experience cyanotic episodes, or "tet spells," that are relieved by squatting. Squatting increases systemic vascular resistance, thus increasing left ventricular (LV) pressure and providing a temporary reversal of the right to left shunt. Diagnosis is confirmed with hematologic and radiologic studies, and treatment options vary from surgical intervention to pharmacotherapy, depending on the severity of symptoms)

A 12-year-old boy was diagnosed with an atrial septal defect (ASD). What would you expect to see on his ECG? A. Left ventricular hypertrophy (LVH) B. Right ventricular hypertrophy (RVH) C. Right bundle branch block (RBBB) D. Left bundle branch block (LBBB)

C (Right bundle branch block (RBBB) (Almost all patients with an atrial septal defect will have an RBBB on their ECG. Left ventricular hypertrophy (LVH) can be seen on conditions such as a ventricular septal defect. Right ventricular hypertrophy and right axis deviation can be seen with tetralogy of Fallot. Left bundle branch block in pediatric patients can be seen in LVH, left ventricular noncompaction, and Wolff-Parkinson-White syndrome)

A 19-year-old basketball player unexpectedly collapses on the court. Several minutes later he returns to consciousness and is able to continue playing. This has happened several times before with similar outcomes. He had no significant past medical history. Which of the following is most likely to be found in this patient? A. Atheromatous plaque rupture B. Coagulation necrosis with loss of nuclei and striations C. Septal hypertrophy D. Postductal coarctation of the aorta E. Cardiac myxoma

C (Septal hypertrophy) (This clinical picture is consistent with hypertrophic cardiomyopathy (HCM) and autopsy would reveal a hypertrophic intraventricular septum) (HCM is the most common cause of sudden cardiac death in otherwise healthy, young athletes. The disorder can be autosomal dominant (>50% cases) or idiopathic. Muscle hypertrophy is asymmetric, and is due to increased synthesis of actin and myosin. On microscopic examination, the cardiac muscle fibers will appear hypertrophied and in disarray. On echocardiography, HCM patients exhibit a septum:left ventricular wall ratio > 1.3:1 and may have systolic anterior motion of the mitral valve with diastolic dysfunction)

A 15-year-old boy comes to the clinic because of progressively worsening dyspnea upon exertion and chest pain for the past 6 months. He denies prior episodes of syncope but describes moments in which he felt lightheaded and weak which improved with rest. Physical examination shows a well-built young man in mild distress. His temperature is 37.5°C (99.5°F), pulse is 65/min, respirations are 17/min, and blood pressure is 110/80 mm Hg. Cardiac examination shows paradoxical splitting of the second heart sound along with a 4/6 systolic crescendo-decrescendo murmur best heard between the apex and the left sternal border on auscultation. Palpation of the precordium leads to a sustained impulse best felt at the apex. Which of the following will most likely decrease the intensity of the murmur heard on the physical examination? A. Diuretics B. Nitroglycerin C. Squatting D. Standing up E. Valsalva maneuver

C (Squatting) (Hypertrophic cardiomyopathy can cause paradoxical splitting of the second heart sound and a systolic ejection murmur on the left side on auscultation) (Given the patient's age and presentation, he most likely has hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy is characterized by a left ventricular outflow tract obstruction caused by hypertrophied interventricular septum in the proximity of the mitral valve. Hypertrophic cardiomyopathy is a familial condition and is also associated with Friedreich's ataxia. Currently, the most common gene associated with hypertrophic cardiomyopathy is a mutation in the beta-myosin heavy chain. On gross pathology, hypertrophic cardiomyopathy shows hypertrophy of all chamber walls and asymmetric septal hypertrophy. Microscopically, the myocardial fibers appear disoriented, tangled, and hypertrophied) (The crescendo-decrescendo murmur on auscultation is caused by a left ventricular outflow tract obstruction on the left side. As a result, the patient is at an increased risk of syncope and sudden death. Any maneuver that increases peripheral vascular resistance would decrease the murmur by decreasing the gradient across the left ventricular (LV) outflow tract. Any maneuver that decreases either preload or afterload would increase the murmur. Squatting decreases the LV outflow gradient by obstructing the femoral artery and hence increasing total peripheral resistance.) (Treatment for hypertrophic cardiomyopathy includes β-blockers and non-dihydropyridine calcium channel blockers such as verapamil)

A 7-year-old boy comes to the emergency department because of worsening shortness of breath for the past 3 months. He has a history of recurrent viral and fungal infections. In the morning, his mother noticed that his lips were bluish in color. He is currently seated leaning forward with his neck extended and has perioral cyanosis and acrocyanosis. His pulse is 120/min, respirations are 44/min, and a blood pressure is 110/80 mm Hg. Cardiac examination shows a 2/6 systolic murmur that worsens with inspiration. Chest radiograph shows an absent thymic shadow, elongated cardiac silhouette, and decreased pulmonary vascular markings. Which of the following diagnoses best describes the mechanism responsible for the patient's shortness of breath and pulmonary findings? A. Coarctation of the aorta B. Eisenmenger syndrome C. Tetralogy of Fallot D. Transposition of the great vessels E. Ventricular septal defect

C (Tetralogy of Fallot) ( The differential diagnosis for cyanosis varies with age and includes coarctation of the aorta, transposition of the great vessels, Tetralogy of Fallot, and Eisenmenger syndrome. Tetralogy of Fallot can be complicated by Eisenmenger syndrome, which presents with cyanosis several years after birth as the initial dynamic shunt becomes essentially right-to-left) (This patient likely has tetralogy of Fallot (TOF), a congenital heart malformation associated with a 22q11.2 deletion syndrome (DiGeorge syndrome). The major characteristics of DiGeorge syndrome include facial abnormalities (cleft lip/palate), cardiac anomalies, and thymus, thyroid, and parathyroid anomalies. The history of viral and fungal infections, along with the absence of a thymic shadow and "boot-shaped" heart on chest radiograph suggests this diagnosis) (The malformations that constitute TOF, one of which is ventricular septal defect (VSD), result in dynamic flow across the VSD dependent on the resistance in the systemic and pulmonary circuits. If systemic resistance is greater than pulmonary resistance, blood will be shunted left-to-right into the pulmonary circulation, and the patient will be acyanotic. If pulmonary resistance is greater, blood will be shunted right-to-left into the systemic circulation, and the patient will be cyanotic. Early in TOF, the former situation predominates; but, as the heart and lungs suffer, resistance to flow on the right side increases and the shunt reverses. This shunt reversal is the essence of Eisenmenger syndrome. The fact that children with ToF experience episodes of transient cyanosis, "Tet spells", further illustrates the dynamic, shifting nature of the initial shunt)

A 2-week-old girl is brought to the emergency department by her mother because of a *blue coloring to her skin.* The mother says that her baby seems "purple," especially her fingers and toes, and looks extremely blue when crying. On physical examination the sleeping baby has *mild cyanosis of the face and trunk,* but *moderate cyanosis of the extremities.* Which of the following is the most common cause of this patient's condition within the first few weeks of life? A. Atrial septal defect B. Patent ductus arteriosus C. Tetralogy of Fallot D. Transposition of the great vessels E. Ventricular septal defect

C (Tetralogy of Fallot) (Tetralogy of Fallot is a congenital heart defect. It is the most common cause of cyanosis within the first few weeks of life. The skin becomes bluish because of the malformed right-to-left shunt. Infants also have worsening cyanosis with agitation, difficulty feeding, and failure to gain weight) (Tetralogy of Fallot is a congenital heart defect that is present at birth. It is the most common cause of cyanosis within the first few weeks of life. The skin becomes bluish because of the malformed right-to-left shunt. Infants also have worsening cyanosis with agitation, difficulty feeding, and failure to gain weight. When affected babies have a bowel movement or cry, they may develop a "tet spell" where they turn blue, become limp, have difficulty breathing, and can lose consciousness. Patients may also have clubbing of the fingers and toes or even polycythemia. Other symptoms may include a heart murmur and easy tiring upon breastfeeding. The four components of the teratology are (1) ventricular septal defect, (2) overriding aorta, (3) infundibular pulmonary stenosis, and (4) right ventricular hypertrophy. Risk factors include a mother who uses alcohol, had diabetes, is over the age of 40, or gets rubella during pregnancy. It is also often associated with Down syndrome. Treatment typically involves open heart surgery during the first year of life)

A male newborn is born at 35 weeks gestation and develops cyanosis of lips and tongue. He is also tachypneic. His temperature is 37ºC (98.6ºF), pulse is 89/min, respiratory rate is 65/min and blood pressure is 80/50 mmHg. Pulse oximetry shows an oxygen saturation of 80% on room air. Physical examination shows perioral cyanosis. Cardiac examination reveals no murmurs and a normal S1 and S2. Following the administration of 100% oxygen the subsequent arterial blood gases reveal a PaO2 of 30 mmHg. Which of the following is the most likely cause of his hypoxemia? A. Tetraology of Fallot B. Arteriovenous fistula C. Transposition of Great Vessels D. Tricuspid Atresia

C (Transposition of Great Vessels) (While a number of problems can be possible for a newborn that results in cyanosis and tachypnea, in most cases, cyanosis can be treated with supplemental oxygen. If supplemental oxygen does not solve the problem, one might suspect a cyanotic heart disease) (The infant in this question is demonstrating characteristic signs and symptoms of cyanotic congenital heart disease, namely transposition of the great arteries (TGA). Cyanotic heart diseases are a group of congenital heart defects that occur due to deoxygenated blood bypassing the lungs and entering the systemic circulation. It is caused by structural defects of the heart. Response to supplemental oxygen in this case is helpful in distinguishing pulmonary from cardiac causes of neonatal hypoxemia. Persistent hypoxemia in the presence of increased oxygen suggests a right-to-left shunt, which is most likely caused by congenital heart disease in the neonatal period)

A 4-year-old Caucasian male suffers from cyanosis and dyspnea relieved by squatting. Which of the following abnormalities is most likely present? A. Left ventricular hypertrophy B. Atrial septal defect C. Ventricular septal defect D. Coarctation of the aorta E. Patent ductus arteriosus

C (Ventricular septal defect) (The patient described is experiencing a "tet spell", characteristic of Tetrology of Fallot (TOF). By definition, TOF consists of 4 components: pulmonic stenosis, ventricular septal defect, right ventricular hypertrophy, and overriding aorta (straddling the VSD).

A 12 year-old boy presents to the office with pain in his legs with activity gradually becoming worse over the past month. He is unable to ride a bicycle with his friends due to the pain in his legs. Examination of the heart reveals an ejection click and accentuation of the second heart sound. Femoral pulses are weak and delayed compared to the brachial pulses. Blood pressure obtained in both arms is elevated. Chest x-ray reveals rib notching. Which of the following is the most likely diagnosis? A. abdominal aortic aneurysm B. pheochromocytoma C. coarctation of the aorta D. thoracic outlet syndrome

C (coarctation of the aorta) (Coarctation is a discrete or long segment of narrowing adjacent to the left subclavian artery. As a result of the coarctation, systemic collaterals develop. X-ray findings occur from the dilated and pulsatile intercostal arteries and the "3" is due to the coarctation site with proximal and distal dilations)

The murmur associated with hypertrophic cardiomyopathy is worsened by conditions that cause ________________ such as the Valsalva maneuver, sudden standing, and tachycardia.

reduced ventricular volume

Patent ductus arteriosus complications?

CHF Endocarditis Pulmonary HTN

Degrees of pulmonary stenosis

Mild/moderate -Asymptomatic -Can progress Severe/Critical -Emergent intervention needed

What is the prevalence of cardiac murmurs in childhood? A. 50% B. 60% C. 70% D. 80%

D (80%) (More than 80% of children have innocent murmurs of one type or another sometime during childhood, usually beginning at approximately 3 or 4 years of age. Half of full-term newborn infants have systolic murmurs sometime during the first week of life. Most of these murmurs are innocent in nature and do not reflect any cardiac pathologic condition)

A 9-year old girl is brought to the clinic by her mother on account of fever. She has red skin lesions on the trunk and proximal extremities, and also small, non-tender lumps located over the joints. On further enquiry, she reports a history of sore throat which occurred about 4 weeks ago. Which of the following would you do to support the diagnosis of acute rheumatic fever? A. Erythrocyte sedimentation rate (ESR) B. Antinuclear antibodies C. White blood cell count D. Anti-streptolysin O titer

D (Anti-streptolysin O titer) (The patient described meets 2 major criteria (Subcutaneous nodules and Erythema marginatum) for the diagnosis of rheumatic fever according to revised Jones criteria. An evidence of a preceding Group A beta hemolytic streptococcus infection is additionally required to make the diagnosis. Elevated ASO titer is an evidence)

A 14-year-old male presents to his primary care physician with complaints of shortness of breath and easy fatigability when exercising for extended periods of time. He also reports that, when he exercises, his lower legs and feet turn a bluish-gray color. He cannot remember visiting a doctor since he was in elementary school. His vital signs are as follows: HR 72, BP 148/65, RR 14, and SpO2 97%. Which of the following murmurs and/or findings would be expected on auscultation of the precordium? A. Mid-systolic murmur loudest at the right second intercostal space, with radiation to the right neck B. Holodiastolic murmur loudest at the apex, with an opening snap following the S2 heart sound C. Left infraclavicular systolic ejection murmur with decreased blood pressure in the lower extremities D. Continuous, machine-like murmur at the left infraclavicular area E. Right supraclavicular continuous murmur which disappears with pressure on the internal jugular vein

D (Continuous, machine-like murmur at the left infraclavicular area) (This patient's presentation of exercise intolerance, lower extremity cyanosis, and a widened pulse pressure is consistent with a diagnosis of patent ductus arteriosus (PDA). A continuous, machine-like murmur loudest at the left infraclavicular area is associated with PDA) (It is important to be able to differentiate PDA from coarctation of the aorta based on examination and history findings. PDA is characterized by lower extremity cyanosis, while coarctation of the aorta manifests with lower extremity exercise intolerance without cyanosis. There is shunting of the blood from the right to left in PDA; in cases of a severe preductal coarctation, a persistent PDA may be necessary to maintain sufficient blood flow (albeit partially venous) to the lower extremities)

seven-month-old male is brought to the pediatrician by his mother because she is concerned that the child's lips occasionally turn blue when he cries. His temperature is 99.2°F (37.3°C), blood pressure is 90/50 mmHg, pulse is 160/min, and respirations are 24/min. On examination, a harsh systemic ejection murmur is heard at the left upper sternal border. A chest radiograph of the infant is shown in Figure A. Which of the following processes most likely contributed to this patient's condition? A. Linear development of the aorticopulmonary septum B. Partial formation of the aorticopulmonary septum C. Excessive resorption of the septum primum D. Displacement of the infundibular septum E. Partial formation of the interventricular septum

D (Displacement of the infundibular septum) (The most likely diagnosis in this patient is tetralogy of Fallot. Tetralogy of Fallot is caused by an anterosuperior displacement of the infundibular septum)

A 30-year-old man comes to the family medicine clinic for an annual physical. On auscultation, a systolic murmur is heard over the left lower sternal border. The murmur is accentuated when the patient stands up from a squatting position and with the valsava maneuver. The murmur decreases in intensity when the patient lays supine. The patient's pulmonary capillary wedge pressure is within normal limits. Which of the following diagnosis is most likely in this patient? A. Aortic dissection B. Aortic regurgitation C. Aortic stenosis D. Hypertrophic cardiomyopathy E. Mitral regurgitation

D (Hypertrophic cardiomyopathy) (Hypertrophic cardiomyopathy is a genetic condition characterized by a systolic murmur which increases in intensity with decreased preload. It is typically asymptomatic until sudden cardiac death. As a result, routine screening of certain populations for this disease is typically suggested) (This patient most likely has hypertrophic cardiomyopathy, a genetic condition that commonly leads to sudden cardiac death in young athletes. Hypertrophic cardiomyopathy leads to enlargement of the interventricular septum. This can lead to left ventricular outflow obstruction during systole due to the motion of the anterior leaflet of the mitral valve. This outflow obstruction leads to the systolic murmur. A decrease in cardiac preload reduces the diameter of the outflow tract and thus increases obstruction which leads to an increased intensity of the murmur. Standing from a squatting position and the Valsalva maneuver are two methods to reduce preload by decreasing venous return)

A 37-year-old man comes to the emergency department because of an episode of syncope at work. He says that he has felt increased shortness of breath while exercising over the past 6 months. He is a well-built man in mild distress. Cardiac examination shows a harsh systolic ejection murmur at the left lower sternal border which is accentuated by forceful expiration against a closed airway. Palpation of the carotid artery shows two closely spaced pulses which occur during systole. Which of the following is the most likely diagnosis? A. Aortic dissection B. Aortic stenosis C. Cardiac tamponade D. Hypertrophic cardiomyopathy E. Third degree heart block

D (Hypertrophic cardiomyopathy) (Hypertrophic cardiomyopathy is a genetic disorder characterized by a crescendo-decrescendo systolic murmur and pulsus bisferiens on physical examination) (Hypertrophic cardiomyopathy is a genetic and inherited form of heart disease. It causes enlargement of the heart wall, in particular, the septum, which leads to left ventricular outflow obstruction. Patients with hypertrophic cardiomyopathy can be characterized by syncope, shortness of breath, and chest pain. The carotid pulse described above is pulsus bisferiens. It has a rapid upstroke followed by a pause as flow reduces. The reduction is due to the left ventricular outflow obstruction and partial closure of the aortic valve that occurs in mid-systole. Lastly, a second pulse is felt which is caused by the tidal wave which is a wave reflected from the periphery. Cardiac examination will also show a loud, harsh, crescendo-decrescendo systolic murmur over the lower left sternal edge with radiation to the axilla. This murmur is accentuated by a Valsalva maneuver or forceful expiration against a closed airway)

An 18-year-old African-American male presents to his family physician for a routine sports physical. He has a family history of sudden death at a young age. Upon physical examination the physician appreciates a systolic murmur. The intensity of the murmur increases when performing a valsalva maneuver. The physician refers the patient for an EKG, which is attached. What is the most likely cause of this murmur? A. Aortic valve stenosis B. Mitral stenosis C. Tricuspid stenosis D. Hypertrophic cardiomyopathy E. Benign systolic flow murmur

D (Hypertrophic cardiomyopathy) (This patient has hypertrophic obstructive cardiomyopathy (HOCM) and has developed a murmur due to abnormal systolic anterior motion of the mitral valve) (HOCM is a significant cause of sudden death in young men, especially if it remains undiagnosed. The systolic murmur often associated with this disease is secondary to dynamic outflow obstruction between the hypertrophied left ventricle and the anterior leaflet of the mitral valve. If the obstruction continues to worsen, surgical intervention may be required to relieve the obstruction and improve cardiac function. A systolic murmur due to HOCM increases in intensity when a valsalva maneuver is performed, a systolic murmur due to aortic valve pathology would decrease in intensity)

A 5-year-old girl presents to the physician with increased muscle cramping in her lower extremities after walking extended distances. The young girl is in the 10th percentile for height. Her past medical history is notable only for a cystic hygroma detected shortly after birth. Which of the following findings is most likely in this patient? A. Decreased blood pressure in the upper and lower extremities B. Barr bodies on buccal smear C. Endocardial cushion defect D. Inferior erosion of the ribs E. Apparent hypertrophy of the calves

D (Inferior erosion of the ribs) (This patient is suffering from an aortic coarctation, which is associated with Turner Syndrome) (Aortic coarctation is a congenital condition that is most often found when the aorta narrows in the region where the ductus arteriosus inserts. The narrowing creates a blood flow/pressure gradient resulting in a decrease in blood flow/pressure to the lower extremities. The vascular system compensates by increasing flow through other vessels including the subclavian and intercostal arteries thus leading to notching of the ribs (erosions from the vasculature). Similar to aortic stenosis due to the narrowing the left ventricle must generate significantly higher pressures to generate blood flow throughout the peripheral circulation. In the long-term this can result in structural changes to the heart)

A 10-year-old male presents with his mother with multiple complaints. A few weeks ago, he had a sore throat for several days that improved without specific therapy. Additionally, over the past several days he has experienced pain in his ankles and wrists and, more recently, his left knee. His mother also noted several bumps on both of his elbows, and he has also had some pain in his center of his chest. He thinks the pain is better when he leans forward. On physical examination, he is noted to be mildly febrile, and a pericardial friction rub is auscultated. Which of the following histopathologic findings is most likely associated with this patient's condition? A. Atypical lymphocytes noted on peripheral blood smear with an initial positive heterophil antibody test B. Plasmodium falciparum ring forms in red blood cells on peripheral blood smear C. Needle-shaped, negatively birefringent crystal deposits surrounded by palisading histiocytes in the synovial fluid of an affected joint D. Interstitial myocardial granulomas containing plump macrophages with nuclei incorporating central wavy ribbons of chromatin E. Sterile vegetations on both the ventricular and atrial aspects of the mitral valve, a proliferative glomerulonephritis, and serum anti-dsDNA and anti-Sm positivity

D (Interstitial myocardial granulomas containing plump macrophages with nuclei incorporating central wavy ribbons of chromatin) (This patient's presentation is consistent with a diagnosis of acute rheumatic fever and associated carditis. Histologic findings associated with this condition include Aschoff bodies, interstitial myocardial granulomas composed of surrounding Anitschkow or caterpillar cells, which are plump macrophages with abundant cytoplasm and central, ovoid nuclei with slender ribbons of chromatin)

At this time the infant with an aymptomatic VSD should" A. have an immediate cardiac catheterization performed, followed by surgical closure within 3 months B. have immediate surgery C. be managed with digoxin and diuretics D. none of the above

D (none of the above) (Watchful expectation should be pursued. The prognosis is excellent, and the defect will probably close spontaneously. As the VSD becomes smaller, the murmur becomes shorter and maintains its regurgitant characteristics (i.e., it starts off with the first heart sound). Spontaneous closure occurs in 30% to 40% of cases, even more frequently in small defects. Pediatric cardiology referral is appropriate for serial examinations and echocardiograms. Infants with a large VSD who develop congestive heart failure and growth retardation should be treated first with digoxin and diuretics. If this fails, surgical intervention is necessary within the first 6 months of life)

An 8 year-old boy is brought to a health care provider complaining of dyspnea and fatigue. On physical examination, a continuous machinery murmur is heard best in the second left intercostal space and is widely transmitted over the precordium. The most likely diagnosis is: A. ventricular septal defect. B. atrial septal defect. C. congenital aortic stenosis. D. patent ductus arteriosus.

D (patent ductus arteriosus)

A 5-day-old boy comes to the emergency department because of altered mental status. His mother called an ambulance after finding him grey and unarousable in his crib. He was born via Cesarean section due to preterm premature rupture of the membranes. Since birth, the infant has gained little weight and has been generally fussy. His temperature is 37ºC (98.6ºF), pulse is 180/min, respirations are 80/min, blood pressure is 50/30 mmHg, and oxygen saturation is 80% on room air. Physical examination shows a mottled, cyanotic infant who is unresponsive to stimulation. Cardiopulmonary examination shows prominent heart sounds, wet rales to the inferior lungs bilaterally, strong brachial pulses but absent femoral pulses. Endotracheal intubation is performed immediately and successfully. Which of the following is the most appropriate next step in management? A. Balloon angioplasty B. Chest radiograph and ECG C. Digoxin D. Intravenous prostaglandin E1 E. Knee-to-chest positioning

D (Intravenous prostaglandin E1) (Coarctation of the aorta is a congenital condition where the aorta is narrow, typically around the area of insertion of the ductus arteriosus. Treatment is an intravenous infusion of prostaglandin E1 to open the ductus arteriosus) (Coarctation of the aorta is a congenital heart defect where the aorta is constricted causing increased flow proximal and decrease flow distal to the constriction. Depending of the size of the constriction, coarctation ranges from being asymptomatic to presenting in overt cardiogenic shock) (This infant is presenting in profound shock: tachycardia, hypotension, tachypnea, hypoxemia and altered mental status. Given the physical exam findings with decreased femoral pulses and signs of heart failure, the most likely cause is a critical coarctation of the aorta which is dependent on a patent ductus arteriosus to maintain circulatory stability. The ductus spontaneously closes within the first few weeks of life, leading to increased volume and pressure on the left ventricle. Eventually the left ventricle fails, leading to cardiogenic shock, as seen in this scenario. Treatment with prostaglandin E1 (PGE1) should be given immediately to maintain patency of the ductus arteriosus after the airway is secured. Remember the ABC's. Intubation will provide an airway and maintenance of breathing. Circulation is critical on recognition of a ductal dependent congenital heart defect and giving PGE1 immediately)

A 15-year-old boy comes to the emergency department because of an episode of syncope during soccer practice. Medical history is noncontributory and he takes no medications. He says he has felt shortness of breath and dizziness after long soccer practices and games over the past month but has not told anyone since he did not want to lose his starting position. His pulse is 70/min and blood pressure is 110/80 mmHg. He is awake, alert, and oriented to person, place, and time. An echocardiogram shows severe left ventricular hypertrophy, a thickened right ventricle, and a prominent interventricular septum. Which of the following is most likely to be associated with his condition? A. Excessive iron accumulation B. GAA triple repeat C. Infection with a single-stranded RNA virus D. Mutation in beta-myosin heavy chain E. Mutation in dystrophin protein

D (Mutation in beta-myosin heavy chain) (Hypertrophic cardiomyopathy is a disease characterized by abnormally thick portions of myocardium. It can result from a mutation in the β-myosin heavy chain) (Hypertrophic cardiomyopathy is a disease in which a portion of the myocardium is hypertrophied without any obvious cause. It is perhaps the most well-known, leading cause of sudden cardiac death in young athletes. Hypertrophic cardiomyopathy often affects the left ventricle and causes increased stiffness and decreased volume within the affected chamber(s), possibly leading to diastolic heart failure. It is hereditary in >70% of cases, follows an autosomal dominant pattern of inheritance, and is associated with mutations in the β-myosin heavy chain. Patients typically present with no prior symptoms and either die unexpectedly usually during exertion or present with symptoms of syncope, chest pain, palpitations, and/or dyspnea during physical activity. Treatment options include beta blockers, calcium channel blockers, or surgical intervention)

An 8 year-old girl is referred to a pediatric cardiologist after a heart murmur was auscultated during a routine school health examination. The patient has not experienced any symptoms of shortness of breath, fatigue, chest pain, or palpitations. She is healthy, does not have any significant medical history, and had an uneventful birth without any complications. Her vital signs are as follows: T 37.2 C, HR 92, BP 104/62, RR 24, SpO2 99%. Physical examination is significant for a midsystolic ejection murmur heard best at the 2nd intercostal space near the left sternal border, a loud S1 heart sound, a widely fixed split S2 heart sound, and no evidence of cyanosis or clubbing. An echocardiogram is conducted and reveals a left-to-right shunt across the interatrial septum. Surgical correction of this patient's condition will most likely prevent which of the following from developing later in life? A. Arteriovenous fistula B. Pulmonary stenosis C. Coronary artery disease D. Pulmonary hypertension E. Aortic root dilation

D (Pulmonary hypertension) (This patient's presentation of a systolic murmur with a widely split, fixed S2 and the above echocardiogram findings is consistent with a diagnosis of atrial septal defect (ASD). Patients with ASD are at risk of developing pulmonary hypertension due to the chronic left-to-right intracardiac shunt)

A 41-year-old male presents complaining of easy fatigability. On physical exam, a wide fixed split S2 and a Grade II/VI systolic murmur is heard over the pulmonic region. Which of the following will be most beneficial in the diagnostic evaluation? A. Obtain an ECG. B. Obtain a chest x-ray. C. Refer for cardiac catheterization. D. Refer for echocardiography.

D (Refer for echocardiography.) (Direct visualization of the atrial septal defect by two-dimensional echocardiography and demonstration of a left to-right shunt through the defect by color-flow Doppler confirms the diagnosis of atrial septal defect)

A newborn is found to be extremely cyanotic immediately after birth. He then develops progressive respiratory failure and is admitted to the neonatal ICU. A single loud S2 heart sound is appreciated as well as a machine-like murmur at the left upper sternal border. Radiography shows an enlarged "egg-shaped" heart. The newborn is then taken for a atrial septostomy to alleviate the condition pending definitive surgical correction. Which of the following is the most likely cause of this newborn's condition? A. Coarctation of the aorta B. Persistent truncus arteriosus C. Tetralogy of Fallot D. Transposition of great vessels E. Tricuspid atresia

D (Transposition of great vessels) (This newborn is presenting with a congenital cyanotic heart malformation most consistent with transposition of the great vessels) (Transposition of the great vessels occurs when the spiral septum does not form correctly leading to a right sided aorta and a left sided pulmonary artery. This causes the pulmonary and systemic circuits to operate in parallel rather than in series. Gas exchange can only occur between the two circuits through a shunt such as a persistent ductus arteriosus or atrial septum defect. These infants are immediately cyanotic and have progressive respiratory failure)

A 10-month-old girl was admitted to the hospital for cardiac catheterization. Her history included cyanosis noted at about 6 weeks of age, increasing over the last 7 months and becoming more severe with crying or physical activity. The chest x-ray demonstrates a "boot shaped heart" A presumptive diagnosis of tetralogy of Fallot (TOF) was made on admission. TOF has 4 components, which of the following below is not part of the diagnosis? A. Pulmonary valve stenosis B. VSD C. Overriding aorta D. Right ventricular hypertrophy E. ASD

E (ASD) (In 1888, Fallot described a congenital heart defect composed of four characteristics (a) large ventricular septal defect (VSD) (b) right ventricular outflow obstruction (pulmonary valve stenosis) (c) overriding aorta (d) right ventricular hypertrophy. The main characteristic of TOF is cyanosis. Hypercyanotic spells or "tet spells" are paroxysmal episodes in which the cyanosis acutely worsens. Crying, feeding, or defecating can bring on these episodes)

A child is found to have higher blood pressures in the arms than in the legs and pulses are bounding in the arms but decreased in the legs. The most likely condition is? A. VSD B. Tetralogy of Fallot C. Transposition of the great arteries D. Aortic stenosis E. Coarctation of the aorta

E (Coarctation of the aorta) (Coarctation of the aorta involves narrowing of the aorta that may occur anywhere along its length, but the vast majority of cases occur just below the origin of the left subclavian artery. The classic clinical sign of coarctation of the aorta is a higher blood pressure in the arms than in the legs and pulses that are bounding in the arms but decreased in the legs. Surgical repair is usually performed between the ages of 2 and 4 years. Emergent surgical repair is performed in cases of circulatory shock, cardiomegaly, severe hypertension, or severe congestive heart failure)

A 34-year-old G1P0 woman gives birth to a male infant at 35 weeks gestation. The child demonstrates a strong cry and moves all his arms and legs upon birth. Respirations are slow and irregular. His temperature is 99.1°F (37.3°C), blood pressure is 100/55 mmHg, pulse is 115/min, and respirations are 18/min. Several hours after the child is born, the nurse notices that the infant's torso and upper extremities are pink while his lower extremities have a bluish hue. Which of the following will most likely be heard on auscultation of the patient's chest? A. Rumbling noise in late diastole B. Early diastolic decrescendo murmur at the left sternal border C. Holosystolic murmur radiating to the right sternal border D. Holosystolic murmur radiating to the axilla E. Continuous systolic and diastolic murmur at left upper sternal border

E (Continuous systolic and diastolic murmur at left upper sternal border) (This patient presents with lower extremity cyanosis with normal upper extremity perfusion suggestive of a patent ductus arteriosus (PDA). A "machine-like" continuous murmur at the left upper sternal border is often heard in patients with PDA)

A 5-day-old infant is brought to the emergency department by his parents because of difficulty breathing and increased fussiness for 24 hours. His parents note that he is sleeping more than usual and has stopped tolerating his feedings. They describe one episode in which his skin appeared blue while crying. He appears lethargic but in no acute distress. Cardiac examination shows bounding pulses and a continuous machine-like murmur. Echocardiography confirms the presence of a patent ductus arteriosus (PDA). Which of the following is the most appropriate next step in management? A. Emergency ligation B. Reassurance and observation overnight C. Antibiotic prophylaxis D. Device closure E. Indomethacin treatment

E (Indomethacin treatment) (Patent ductus arteriosus can be treated initially by medical management with indomethacin, which promotes closure by inhibiting prostaglandin synthesis. If medical management fails, surgical intervention by ligation or percutaneous occlusion may be indicated) (After a newborn takes the first breath, the lungs expand, causing decreased pulmonary vascular resistance (PVR) and release of bradykinin that results in smooth muscle constriction in the wall of the ductus arteriosus. This constriction reduces blood flow through the ductus arteriosus, leading to narrowing and spontaneous closure. In normal development, the ductus arteriosus is mostly closed within 1-2 days and completely closed within 3 weeks. Patent ductus arteriosus (PDA) is the lack of spontaneous postnatal closure, leading to left-to-right shunting from the aorta to the pulmonary artery. Factors contributing to PDA closure are increases in O2 content and withdrawal of maternal prostaglandins, including prostaglandin E1.Treatment of PDA depends on the size of the defect. A small PDA may be asymptomatic and typically closes spontaneously. Children with moderate or large PDAs are characterized by poor feeding, exercise intolerance, increasing fatigue, wide pulse pressure, and a continuous machine-like murmur heard best over the left infraclavicular area. Initial management includes intravenous indomethacin, which inhibits synthesis of prostaglandins that promote continued patency. For larger defects, or those that do not respond to medical treatment, surgical repair by ligation or percutaneous occlusion may be indicated, typically within the first 6 months of life)

The most common location for an atrial septal defect is a _______________________.

Patent foramen ovale

A_____________, ___________ or an _____________must be present in anomalous pulmonary venous connection for the individual to survive.

Patent foramen ovale Patent ductus arteriosa Atrial septal defect

A 4-year-old boy with a history of a failure to thrive comes to the office for an annual check up. The patient's mother says that after his first year of life. the boy has generally been developing well, although she has occasionally observed him becoming short of breath while playing outside with his friends, and that he frequently squats down on his knees when he is winded. His mother denies any other medical problems or surgeries. During the visit, the boy starts to complain that he doesn't feel well and wants to go home. During physical examination. develops perioral cyanosis and collapses on the floor. Which of the following is the most appropriate immediate intervention in the management of the boy's condition? A. Administering 0.5 mg dose of intravenous atropine B. Administering a 0.5 mg/kg dose of intravenous diazepam C. Administering a 360 J shock using an automatic external defibrillator D. Performing an emergency tracheotomy E. Placing the child on his side and bringing his knees up to his chest

E (Placing the child on his side and bringing his knees up to his chest) (Tet spells due to tetralogy of Fallot can be treated by having the patient lay on their side and bringing their knees up to their chest to increase pulmonary blood flow) (Tetralogy of Fallot (TOF) is a congenital heart defect characterized by pulmonary stenosis, right ventricular hyperplasia, an overriding aorta and a ventricular septal defect (VSD). Cyanosis in TOF is due to right-to-left shunting across the VSD that occurs secondary to the increased pressure in the right ventricle (RV), caused by the stenotic pulmonary valve. The episodes of shortness of breath that are relieved by squatting are characteristic of the cyanotic episodes or "tet spells" that these patients can experience, and can be thought of as an imbalance between pulmonary and systemic vascular resistance that favors increased right-to-left shunting and decreased pulmonary blood flow. Severe tet spells can lead to sudden cyanosis and syncope. The cause of TOF is unknown; however, several genetic and environmental factors may play a role. The emergency management of tet spells includes placing the patient on their side and bringing the patient's knees to their chest, which will increase systemic vascular resistance and decrease the right-to-left shunting, thereby increasing pulmonary blood flow)

A 16-year-old boy comes to the emergency department because of a sudden loss of consciousness during a high school basketball game. Despite appropriate resuscitation protocol, the patient is later pronounced dead. The patient's medical history is noncontributory. His mother says he has a cousin who died in his twenties due to a "big heart." An autopsy is performed. Which of the following findings will be most likely found upon gross examination of the patient? A. Accumulation of pericardial fluid B. Left atrial myxoma C. Occlusion of the left anterior descending artery D. Ruptured chordae tendineae E. Thickened interventricular septum

E (Thickened interventricular septum) (Hypertrophic cardiomyopathy is an autosomal dominant genetic condition characterized by a mutation in the cardiac sarcomere protein leading to a thickened interventricular septum) (Hypertrophic cardiomyopathy is an autosomal dominant genetic condition characterized by a mutation in the cardiac sarcomere protein. This leads to hypertrophy of the interventricular septum of the heart. Microscopic examination reveals a disorganized and thickened collagen matrix. The thickened and hypertrophied septum leads to narrowing of the left ventricular outflow tract. During systole, the outflow tract is further obstructed secondary to the inward movement of the interventricular septum and this is the mechanism of sudden cardiac death in this disease. Seventy-one percent of patients who die suddenly have no or mild symptoms and only fifteen to twenty percent were participating in moderate to vigorous physical activity at the time of death. Physical examination can show no abnormalities, but findings consistent with hypertrophic cardiomyopathy include a crescendo-decrescendo systolic murmur accentuated by the valsalva maneuver, pulsus bisferiens, and a sustained impulse palpated at the apex)

A 25-year-old female is diagnosed with hypertension by her primary care physician after being found to have persistently elevated blood pressure measurements. She also has a history of intermittent headaches and also complains of leg cramps after walking. On physical exam she is found to have a systolic heart murmur as well as cold feet with a poor capillary refill time. In addition, a blood pressure from her lower extremity is found to be significantly lower than in her upper extremities. Based on clinical suspicion, a chest radiograph is obtained and reveals notching of the ribs. Which of the following clinical conditions is associated with this patient's most likely disorder? A. Down syndrome B. Infection with Streptococcus pyogenes C. Marfan syndrome D. Systemic lupus erythematosis E. Turner syndrome

E (Turner syndrome) (The most likely diagnosis in this case is coarctation of the aorta based on brachial-femoral delay and rib notching on chest radiograph. Turner syndrome is associated with a higher risk of coarctation)

A 33-year-old G2P1 woman comes to the office because of poor diabetic control. She is currently at 18 weeks' gestation and admits to having poor control of her type I diabetes before becoming pregnant. Family history is non-contributory. Examination shows a pregnant woman with a fundal height of 20 cm (7.9 in). An abdominal ultrasound is ordered. Which of the following is the most likely congenital abnormality shown on the ultrasound? A. Amelia B. Anencephaly C. Sacral agenesis D. Spina bifida E. Ventricular septal defect

E (Ventricular septal defect) (Ventricular septal defect is a congenital abnormality of cardiac origin. Cardiac anomalies are associated with poorly controlled gestational diabetes) (Infants of diabetic mothers (IDMs) have experienced a nearly 30-fold decrease in morbidity and mortality rates since the development of specialized maternal, fetal, and neonatal care for women with diabetes and their offspring. Before then, fetal and neonatal mortality rates were as high as 65%) (Common abnormalities in IDMs are growth abnormalities (large for gestational age [LGA], small for gestational age [SGA]), hyperviscosity secondary to polycythemia, and hypoglycaemia) (The most common congenital organ anomaly associated with poorly controlled pregestational diabetes are cardiac anomalies. The most common cardiac anomaly is a ventricular septal defect (VSD), which can be seen on foetal ultrasound. Management of VSDs is variable depending on their size)

A 3-year-old boy who was recently diagnosed with a congenital heart defect, comes to his pediatrician's office for a follow-up visit. Two months ago, he was referred to a pediatric cardiologist after his parents noticed that he seemed to be in distress frequently, especially after running around the house. He would also squat down to catch his breath. The pediatric cardiologist informed the parents that the squatting helps to compress the femoral arteries which increases systemic blood pressure, reducing the right-to-left blood shunting in his heart. Which of the following heart sounds is most likely to be auscultated in this patient due to his condition? A. Fixed splitting of S1 B. Fixed splitting of S2 C. Normal splitting of S2 D. Paradoxical splitting of S2 E. Wide splitting of S2

E (Wide splitting of S2) (Wide splitting of S2 is a finding in the tetralogy of Fallot, and it is primarily caused by stenosis of the pulmonic artery/valve) (Tetrology of Fallot (TOF) is a congenital heart defect characterized by pulmonary stenosis, right ventricular hyperplasia, an overriding aorta and a ventricular septal defect (SVD). Cyanosis in TOF is due to right-to-left shunting across the VSD that occurs secondary to the increased pressure in the right ventricle (caused by the stenotic pulmonary valve). The episodes of shortness of breath that are relieved by squatting are characteristic of the cyanotic episodes or "tet spells" that these patients can experience, and can be thought of as an imbalance between pulmonary and systemic vascular resistance that favors increased right-to-left shunting and decreased pulmonary blood flow. On auscultation, wide splitting of S2 is most commonly seen in pulmonic stenosis due to the lengthening of right ventricular ejection time)

Diagnosing CHD

Echocardiography: fetal and neonatal Microvascular cardiac catheterization Fetal screening Neonatal pulse oximetry screening

What can be used to diagnose suspected congenital heart defects in children?

Echocardiography: fetal and neonatal Microvascular cardiac catheterization Fetal screening Neonatal pulse oximetry screening

Diagnostic Evaluation

History Physical examination CXR ECG Echocardiogram Cardiac catheterization Imaging/Recording

What type of murmur is heard with an AV Septal Defect?

Holosystolic murmur

A 25-year-old woman presents the ER after a syncopal episode. She had loss of consciousness 3 times over the past 12 months. Each event occurred during or just after physical exercise. On PE: BP 110/70 mm Hg, HR 75/min, normal S1/S2, and an III/VI systolic ejection murmur is heard best at the left sternal border that decreases with squatting. The EKG shows a normal sinus rhythm with diffuse increased QRS voltage. What is the most likely diagnosis?

Hypertrophic cardiomyopathy

What is the most common cause of death from cardiac defects in the first month of life?

Hypoplastic Left Heart Syndrome

Shock may be seen in which types of CHD?

Hypoplastic Left Heart Syndrome Critical Aortic Stenosis Critical Coarctation of Aorta Interrupted Aortic Arch

EKG of Atrial septal defect?

IRBBB or RBBB.

Truncus Arteriosus repair

Mobilize pulmonary arteries and reattach to right Ventricle to the pulmonary artery conduit Open and repair truncus with pericardial patch Closure of ventricular septal defect

Functional or pathological murmur? Diastolic Harsh Loud

Pathologic murmur

You should suspect a congenital heart disease if you see what?

Pathologic murmurs Abnormal heart rate Diminished peripheral pulses BP >10mm Hg higher in arms than legs Cyanosis Respiratory Distress Shock Abnormal pulse oximetry screen

Functional or pathological murmur? Diastolic Harsh Loud

Pathological murmur

(Coarctation of the aorta / Patent ductus arteriosis) is characterized by lower extremity cyanosis, while (Coarctation of the aorta / Patent ductus arteriosis) manifests lower extremity exercise intolerance without cyanosis.

Patent ductus arteriosis; Coarctation of the aorta

A 2-week-old infant with a history of prematurity presents with a pink torso and upper extremity and blue lower extremities. On cardiac auscultation, you notice a rough, continuous "machinery murmur" heard over the left sternal border at the 2nd intercostal space (pulmonary area). What is the most likely diagnosis?

Patent ductus arteriosus

The transient systolic murmur of_____________ is audible at the upper left sternal border and in the left infraclavicular area on the first day, and it usually disappears soon thereafter.

Patent ductus arteriosus

What defect? Common lesion, associated with many defects and syndromes including Down's Volume load to left ventricle with left ventricular enlargement and left atrial enlargement Continuous murmur

Patent ductus arteriosus

_____________________is a persistence of the fetal connection (ductus arteriosus) between the aorta and pulmonary artery after birth, resulting in a left-to-right shunt.

Patent ductus arteriosus

A 9 lb male newborn is delivered at term to a gravida 2, para 2, mother. *Maternal medical history includes poorly controlled type I diabetes mellitus.* Prenatal ultrasound shows that the *baby has hypoplastic left heart syndrome* (HLHS). *Which of the following findings must the patient also have to ensure initial survival?*

Patent ductus arteriosus Atrial septal defect

In Hypoplastic Left Heart Syndrome survival is dependent on:

Patent ductus arteriosus for systemic circulation and atrial septal defect for mixing of blood.

What is the result of Hypoplastic Left Heart Syndrome?

Right ventricle supports both pulmonary and systemic circulation.

Characteristics of Tetralogy of Fallot

Right ventricular Outflow Tract Obstruction Ventricular Septal Defect Deviation of origin of Aorta to the right so that it Overrides the VSD Concentric Right Ventricular Hypertrophy

How to Recognize a Suspected Cardiac Defect in a Neonate through clues in the history?

Risk factors Poor feeding Color changes Excessive irritability Excessive sweating Poor weight gain Excessive sleeping

Occurs when O2 saturation drops suddenly due to constriction of pulmonary outflow with more blue blood shunted into aorta and out to body

Tetrology spell

In most cases, ventricular septal defects are defects of the (membranous/muscular) portion of the interventricular septum.

membranous

Atrial septal defect is a (cyanotic/ acyanotic) congenital heart disorder.

acyanotic

Pulmonary veins connect to various anomalous locations (left innominate vein, SVC, IVC, coronary sinus) The right atrium receives blood from both pulmonary and systemic venous systems resulting in Right Atrial Enlargement and Right Ventricular Enlargement.

Total Anomalous Pulmonary Venous Return (TAPVR)

Rare cyanotic congenital heart defect in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation.

Total Anomalous Pulmonary Venous Return (TAPVR)

What malformation? Mixed blood is shunted right to left at atrial level (PFO) causing cyanosis Difficult echo Not ductal dependent Surgical emergency

Total Anomalous Pulmonary Venous Return (TAPVR)

A mother brings her 3-week old infant to the pediatrician's office because he was *not back to birth weight at the baby's initial visit* or 2-week weight check, and she is concerned he is *not feeding well*. PE shows the infant is *tachypnic, diaphoretic,* and *lethargic*. He also has a *cleft palate*. Based on this information, which of the following is the most likely cause of this patient's presentation?

Truncus Arteriosus

What heart deformity? Common arterial trunk which gives rise to aorta and pulmonary arteries. Often have aortic arch abnormalities and Ventricular Septal Defect. 22q11.2 deletions

Truncus Arteriosus

What heart deformity? DiGeorge syndrome

Truncus Arteriosus

A bicuspid aortic valve and coarctation of the aorta processes associated with ______________ syndrome.

Turner syndrome.

In cases of severe pulmonary hypertension due to a ventricular septal defect, surgical repair is (indicated/contraindicated) .

contraindicated

What degree of Coarctation of the Aorta? Ductal dependent systemic blood flow Low cardiac output Differential cyanosis

critical

Transthoracic ________________________is the best diagnostic tool to confirm aortic coarctation and to rule out associated cardiac anomalies.

echocardiography

The murmur of TOF is classically described as a harsh systolic ejection murmur heard best at the___________________

left sternal border

Transposition of the great vessels is characterized by the pulmonary artery arising from the ___________ and the aorta arising from the right ventricle.

left ventricle

An atrial septal defect initially presents with a (right-to-left/left-to-right) shunt.

left-to-right

Patent ductus arteriosus causes a (right to left/left to right) shunt.

left-to-right

There is a (right to left/left to right) shunt between the atria in hypoplastic left heart syndrome.

left-to-right

Infants with (dextro-transposition of the great vessels/levo-transposition of the great vessels) might not show symptoms at birth, but will eventually develop heart failure later in life.

levo-transposition of the great vessels

Normal closure of the ductus arteriosus results in the formation of the _______________________.

ligamentum arteriosum

Atrial septal defect presents with a (soft/loud) first heart sound.

loud

In hypoplastic left heart syndrome, the second heart sound is (loud/muffled).

loud

A (high/low) oxygen tension maintains the patency of the ductus arteriosus.

low

Uncorrected patent ductus arteriosus can eventually result in cyanosis in the (upper/lower) extremities.

lower

Carditis is a (major/minor) criterion in rheumatic fever diagnosis.

major

Erythema marginatum is a (major/minor) criterion in rheumatic fever diagnosis.

major

Subcutaneous nodules are a (major/minor) criterion in rheumatic fever diagnosis.

major

Sydenham's chorea is a (major/minor) criterion in rheumatic fever diagnosis.

major

Neonates with patent ductus arteriosus who weigh less than five kilograms are best managed (surgically/medically) .

medically

A prolonged PR interval on ECG is a (major/minor) criterion in rheumatic fever diagnosis.

minor

Fever is a (major/minor) criterion in rheumatic fever diagnosis.

minor

Inactive heart disease or previous history of rheumatic fever are (major/minor) criteria in rheumatic fever diagnosis.

minor

Joint pain without swelling, also called arthralgia, is a (major/minor) criterion in rheumatic fever diagnosis.

minor

Leukocytosis is a (major/minor) criterion in rheumatic fever diagnosis.

minor

Raised erythrocyte sedimentation rate and C-reactive protein are (major/minor) criteria in rheumatic fever diagnosis.

minor

An atrial septal defect has (missing/unfused) septal tissue, in contrast to a patent foramen ovale.

missing

Coarctation of the aorta is associated with ____________ of the ribs on an x-ray due to collateral circulation.

notching

Hypertrophic cardiomyopathy has a thick interventricular septum that is too close to the anterior mitral leaflet, which causes ________________.

outflow obstruction

The four features of tetralogy of Fallot are pulmonary stenosis, right ventricular hypertrophy, a(n) ventricular septal defect, and an _______________.

overriding aorta

What is O in PROV for Tetrology of Fallot?

overriding aorta

What is a complication of atrial septal defect in which a clot from a deep vein thrombosis can cross over from the right heart to the left causing stroke.

paradoxical embolus

Squatting increases the _____________________ which diminishes the right-to-left shunt and increases pulmonary blood flow.

peripheral vascular resistance

In tetralogy of Fallot, there is a delayed (aortic/pulmonic) component to the S2 heart sound.

pulmonic

The most common cause of mitral stenosis is ______________.

rheumatic fever

The (right/left) ventricle is hypertrophied in the tetralogy of Fallot.

right

In Eisenmenger syndrome, the ventricular septal defect causes (right to left/left to right) right to left shunt.

right to left shunt

Absence of a tricuspid valve leads to a hypoplastic (undersized) or absent _______________ .

right ventricle

In tricuspid atresia, the (left/right) ventricle becomes hypoplastic.

right ventricle

Transposition of the great vessels is characterized by the pulmonary artery arising from the left ventricle and the aorta arising from the _______________

right ventricle

The four features of tetralogy of Fallot are pulmonary stenosis, ______________, a ventricular septal defect, and an overriding aorta.

right ventricular hypertrophy

What is R in PROV for Tetrology of Fallot?

right ventricular hypertrophy

There is a (left-to-right / right-to-left) shunt involved in a persistent truncus arteriosus.

right-to-left

There is a (right to left/left to right) shunting of blood through the patent ductus arteriosus in infantile coarctation of the aorta.

right-to-left

Tricuspid atresia causes a (left-to-right/right-to-left) shunt.

right-to-left

There is a (right to left/left to right) in tetralogy of Fallot.

right-to-left shunt

Atrial septal defect (ASD) is an opening in the interatrial septum, causing a left-to-right shunt and volume overload of the (left / right) atrium and (left / right) ventricle.

right; right

Chest x-ray for an Atrial Septal Defect shows cardiomegaly with dilatation of the (left / right) atrium and (left / right) ventricle, a prominent main pulmonary artery segment, and increased pulmonary vascular markings

right; right

With a significant Atrial Septal Defect shunt, ECG may show (left / right) axis deviation, (left / right) ventricular hypertrophy, or right bundle branch block (with a rSR′ pattern in V1 ).

right; right

Atrial septal defect is a congenital heart disorder that is associated with wide fixed splitting of the (first/second) heart sound.

second

What kind of shock needs to be ruled out?

septic

The murmur associated with hypertrophic cardiomyopathy is worsened by conditions that cause reduced ventricular volume such as the Valsalva maneuver, sudden _____________, and tachycardia.

standing

Coarctation of the aorta involves narrowing of the aorta that may occur anywhere along its length, but the vast majority of cases occur just below the origin of the left _____________ artery.

subclavian

The murmur associated with hypertrophic cardiomyopathy is worsened by conditions that cause reduced ventricular volume such as the Valsalva maneuver, sudden standing, and ________________.

tachycardia

Hypertrophic cardiomyopathy is a cardiomyopathy that is often the cause of sudden cardiac death in young athletes due to __________________.

ventricular arrhythmia

Most individuals with a persistent truncus arteriosus have an accompanying ________________.

ventricular septal defect

The four features of tetralogy of Fallot are pulmonary stenosis, right ventricular hypertrophy, a ______________, and an overriding aorta.

ventricular septal defect

Autosomal dominant mutations in_________________are the most common cause of hypertrophic cardiomyopathy.

β-myosin heavy chains