PA BOARDS - CONGENITAL HEART DISEASE

Acyanotic Congenital Heart Disease:

1. ASD: fixed split S2 2. VSD: most common congenital heart defect, harsh holosystolic murmur at LLSB, complication: Eisenmenger syndrome 3. PDA: machine-like murmur, Rx: indomethacin 4. Coarctation of the aorta: upper extremity > lower extremity BP/pulse, CXR: rib notching, Rx: PGE1 Dx: Echocardiography

Causes a fixed widely split S2

ASD

Signs and Symptoms: These children are usually asymptomatic, but over time can lead to heart failure or recurrent respiratory infections.

ATRIAL SEPTAL DEFECT

Signs and Symptoms: You will hear a systolic ejection murmur with a fixed wide splitting of S2. Patients are at risk for paradoxical emboli leading to stroke.

ATRIAL SEPTAL DEFECT

This is a direct connection between both atria through a defect in the septum.

ATRIAL SEPTAL DEFECT

How does one differentiate a pulmonary versus cardiac source of cyanosis?

Administration of oxygen to cyanotic infants with pulmonary disease typically raises pulse oximetry to 95% whereas in cardiac disease the oxygen saturation will likely remain below 90%.

Management of spells with Tetralogy of Fallot:

Aims to increase systemic vascular resistance, correct hypoxia and correct acidemia. Initial steps include administering high-flow oxygen by mask, consoling the child by cradling them in a knee-chest position, and avoiding exacerbating distress. Underlying arrhythmia, hypothermia, and hypoglycemia should also be corrected.

What medication and dose can be used to temporarily preserve patency of the ductus arteriosus?

Alprostadil (prostaglandin E1), 0.05-0.1 mcg/kg/min intravenously.

Which congenital heart diseases cause right ventricular hypertrophy?

Atrial septal defect, Eisenmenger's syndrome and pulmonic stenosis.

Congenital heart defect associated with Down's Syndrome

Atrioventricular Septal Defect

What is the most common inheritance pattern of dilated cardiomyopathy?

Autosomal dominant.

Closure of the ductus arteriosus begins when levels of which of the following substances increases in the immediate post-natal period?

Bradykinin: After the first breath, pulmonary vascular resistance decreases and stimulates bradykinin release from the infant's lungs. This causes smooth muscle contraction about the ductus arteriosus, beginning the narrowing process until this structure scars close to become the ligamentum arteriosum.

Diagnostic Testing: Diagnosed by echocardiogram. Chest x-ray will show rib notching or a "3" sign at the site of coarctation.

COARCTATION OF THE AORTA

Narrowing of the aorta at the ductus arteriosus.

COARCTATION OF THE AORTA

Signs and Symptoms: May present with hypertension or respiratory distress. There will be a reduced blood pressure and a reduced pulse in the lower extremities.

COARCTATION OF THE AORTA

Treatment: These are surgically corrected.

COARCTATION OF THE AORTA

BP in arms > legs, "rib notching"

Coarctation of Aorta

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.

Coarctation of the aorta

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.

Coarctation of the aorta

In older children, the ECG and chest x-ray usually show left ventricular hypertrophy and a mildly enlarged heart. Rib notching may also be seen in older children >8 years old with large collaterals.

Coarctation of the aorta

Which of the following is most likely to present as a ductal-dependent cardiac lesion?

Coarctation of the aorta

Name one complication of ventricular septal defect corrective-surgery?

Conduction defects, such as transient right bundle branch block.

Congenital cardiac defects occur in 8 out of 1000 live births. Up to one third of infants born with a congenital cardiac defect develop life-threatening symptoms within the first few days of life, with 80% of infants presenting with:

Congestive heart failure: pulmonary or peripheral edema or both. The mortality rate in this critical period is 90%. The majority of these defects can be screened for in the prenatal period with four-chamber echocardiography. Consider screening in women with diabetes, a family history of congenital heart disease, indomethacin exposure or rubella exposure.

A 12 month-old child with tetralogy of Fallot is most likely to have which of the following clinical features?

Cyanosis: very common in tetralogy of Fallot

Which of the following congenital heart diseases would benefit from palivizumab administration?

Cyanotic congenital heart disease

Diagnostic Testing for VSD:

Diagnosed with echocardiogram.

Diagnostic Testing of PDA:

Diagnosis is made with echocardiogram.

A patent ductus arteriosus is actually beneficial in infants with which other congenital heart diseases?

Ductal dependent lesions include transposition of the great vessels, Tetralogy of Fallot, tricuspid atresia, hypoplastic left heart and severe coarctation of the aorta.

A 24-year-old pregnant woman presents to the urgent care clinic where you are working. During your assessment, she states that she saw her obstetrician when she first became pregnant but has not had any follow-up care since. You also find out that she has bipolar disorder and took lithium throughout the first trimester. What congenital abnormality is the fetus at risk of developing?

Ebstein's anomaly: a congenital cardiac malformation that can in part be attributed to maternal lithium use during pregnancy, especially within the first trimester.

What syndrome is associated with a VSD that results in right-to-left shunting?

Eisenmenger syndrome. Progressive high pulmonary vascular pressure alters flow from left to right through the VSD to right to left, leading to cyanosis.

In which of the following conditions is hypoxemia caused by a right-to-left shunt?

Eisenmenger's syndrome: this occurs when increased pulmonary blood flow from a left-to-right shunt leads to pulmonary hypertension and compensatory right ventricular hypertrophy, and, over time, right ventricular pressures surpass left ventricular pressures, resulting in a change in direction of the shunt.

What is the most common cause of pediatric hypertension?

Essential, or primary, hypertension is most common in both adults and pediatrics.

A 3-year-old boy is referred to the Emergency Department due to concern for Kawasaki Disease. He has persistent high fevers for five days. He also has a generalized scarlatiniform rash, red and cracked lips, and three days of watery diarrhea. Today he developed bilateral conjunctival injection without exudate. In addition to the features described, generalized lymphadenopathy is also noted. Which of this patient's clinical features is inconsistent with a diagnosis of Kawasaki Disease?

Generalized lymphadenopathy is not typical of Kawasaki Disease and should prompt consideration of alternative infectious, rheumatologic, or oncologic etiologies. The lymphadenopathy of Kawasaki Disease is typically confined to the anterior cervical region and is almost always unilateral.

What unique GI finding is associated with Kawasaki disease?

Hydrops of the gallbladder; which is characterized by RUQ pain, jaundice, and boggy gallbladder on abdominal ultrasound.

Treatment to close PDA:

Indomethacin, a prostaglandin inhibitor.

Which chromosome abnormality is Tetralogy of Fallot associated with?

It is associated with chromosome 22 deletions and DiGeorge syndrome.

#1 cause of pediatric acquired heart disease: The peak incidence is between the ages 18 and 24 months, with the majority of patients <4 years old and boys are affected more frequently than girls.

Kawasaki Disease: Asians < 4 years old; CRASH and burn: Conjunctivitis, Rash: non-vesicular, no bullae, Adenopathy: cervical, unilateral, Strawberry tongue, Hand/feet edema, fever; Rx: IVIG + aspirin

A six-year-old girl is brought by her mother to the clinic due to fever and sore throat. The girl has been having a fever for five days with a maximum temperature of 38.5°C that is minimally responsive to antipyretics. She is irritable with decreased appetite. On examination there is bilateral bulbar injection, erythema of oral mucosa, enlarged anterior cervical nodes and macular rash on trunk and extremities. Which of the following is the most likely diagnosis?

Kawasaki disease

Symptomatic PDA is treated surgically, with ______ being the most preferred method of closure.

Ligation

Endocarditis can be reduced in patients with ventricular septal defects with which non-pharmaceutical practice?

Maintaining good oral hygiene.

The patient in this vignette has a ductal-dependent cardiac lesion that requires a PDA to preserve blood flow from the aorta to the pulmonary artery. At approximately 1 week of age, the PDA may close and cause sudden cardiovascular collapse. Prostaglandin E1 = PGE1 infusion is potentially life-saving, but it is important to be aware of its adverse reactions, which include:

Many of the adverse effects of PGE1 are dose-related. Apnea, flushing, fever, bradycardia, and/or hypotension may indicate excessive prostaglandin effect and the need for dose reduction.

A 7-year-old boy presents for a well child check. His mother reports that he has always been a healthy child and does well in school. On examination, he is noted to have pectus excavatum and long, tapered fingers. He is wearing glasses, and mother reports a history of upward lens subluxation. What is the most likely genetic abnormality?

Marfan syndrome

Does a Still's murmur radiate to the carotids?

No.

Palivizumab should be administered monthly prior to the onset of the peak RSV season. When is the peak of RSV season typically?

November through March.

The ductus arteriosus is a connection between the main pulmonary artery and the aorta. It usually closes the first two days of life. The ductus arteriosus is kept open by a low oxygen environment and prostaglandins. When the oxygen levels rise - the PDA will close. Failure to close is termed ______.

PATENT DUCTUS ARTERIOSUS

"Machinery murmur"

PDA

Classically described in children as a continuous machinery-type murmur heard best in the second left intercostal space and is widely transmitted across the precordium.

PDA

Patient will be complaining of failure to thrive, poor feeding, tachycardia, and tachypnea PE will show continuous, rough, "machinery-like" murmur, heard best in the first interspaces of the LSB

PDA

Signs and Symptoms: You will hear a machine like continuous murmur.

PDA

Physical activity limitations may be recommended for children with a past history of congenital heart disease. There are no restrictions on any sport activity, including competition and contact sports, in patients who have had surgical correction of tetralogy of Fallot & whose right ventricular pressure is < 50 mm Hg.

Physical activity limitations may be recommended for children with a past history of congenital heart disease. There are no restrictions on any sport activity, including competition and contact sports, in patients who have had surgical correction of tetralogy of Fallot and whose right ventricular pressure is < 50 mm Hg.

What drug can be administered to reopen a recently closed ductous arteriosus?

Prostaglandin E1.

Palivizumab is a humanized monoclonal antibody administered intramuscularly to prevent respiratory syncytial virus or RSV infections.

RSV is the most common cause of lower respiratory tract infections in infants and children worldwide. Children with congenital heart disease (CHD) who develop RSV infections tend to have a higher rate of ICU admissions and require mechanical ventilation more frequently than do children without CHD.

Those with surgically repaired tetralogy of Fallot, with right ventricular pressure > 50 mm Hg or cardiomegaly, should not participate in:

Rigorous or contact sports. However, they may play leisure sports such as jogging, golf or cycling.

What abnormal heart sounds would be heard with Ebstein's anomaly?

Split S2 and a systolic murmur. Associated with Tricuspid Regurgitation.

An asymptomatic 4-year-old boy presents for a routine check-up. On examination you hear a systolic heart murmur that is heard best in the lower precordium and has a low and short tone. It does not radiate and decreases in intensity with inspiration. The remainder of the examination is normal. Which one of the following is the most likely diagnosis?

Still's Murmur: a benign murmur of childhood. It can occur at any age, but is most commonly heard between ages 2 and 8 years. The murmur usually resolves spontaneously by adulthood. It has no association with physiologic or anatomic abnormalities. It is low-grade mid-systolic murmur localized near the apex to lower left sternal border. It decreases in intensity with inspiration, sitting up, or standing.

Diagnostic Testing: May see a boot shaped heart on CXR. Diagnosed with echocardiogram.

TETRALOGY OF FALLOT

Signs and Symptoms: Harsh systolic ejection murmur heard best at the left sternal border.

TETRALOGY OF FALLOT

Signs and Symptoms: Tet spells: hyper-cyanotic episodes, lips, that develop during crying or feeding. Bringing the child's knees to the chest will increase vascular resistance which will in turn make the child more comfortable.

TETRALOGY OF FALLOT

Treatment: Treated by surgical repair.

TETRALOGY OF FALLOT

Cyanotic heart disorder that is characterized by: •Right ventricular hypertrophy •Overriding aorta •Pulmonary artery stenosis = Right ventricular outflow obstruction = narrowing of the pulmonary valve •VSD

TETRALOGY OF FALLOT = "ROP-V"

"Boot shaped heart" on CXR

Tetralogy of Fallot

A newborn is being evaluated for cyanosis. Physical examination shows a prominent right ventricular impulse, a systolic thrill, and clubbing of the fingers and toes. A crescendo-decrescendo murmur with a harsh systolic ejection quality is heard along the left upper sternal border. Based on these findings, which of the following is the most likely diagnosis?

Tetralogy of Fallot

Most common cyanotic congenital heart disease

Tetralogy of Fallot

Treatment of Atrial Septal Defect:

The ASD will usually close spontaneously, but surgery may be performed at age 4 or in those who are severely symptomatic.

Diagnostic Testing for ASD:

The best test for diagnosis is an echocardiogram.

What feature of tetralogy of Fallot determines the prognosis?

The prognosis of tetralogy of Fallot depends on the severity of right ventricular outflow tract obstruction.

The typical flow of blood across an atrial septal defect occurs in which of the following fashions?

This condition initially results in a left-to-right shunt as oxygenated blood from the higher-pressure left atrium passes into the right atrium. This congenital defect can go undiagnosed for 40-50 years, as it is typically asymptomatic. In fact, the majority of cases are diagnosed in the fifth decade. Over time, a large defect may lead to pulmonary overcirculation, pulmonary hypertension, Eisenmenger syndrome, right-to-left shunting, and cyanosis. When symptomatic, patients complain of fatigability, exertional dyspnea or recurrent respiratory infections.

"Egg on a string"

Transposition of Great Vessels

Ductal Dependent Lesions:

Transposition of the great vessels: most common cause in newborns). Tetralogy of Fallot: most common in children >1 year old. Tricuspid atresia, Interrupted aortic arch. Coarctation of the aorta, Hypoplastic left heart syndrome, Shock, "gray baby" within hours to days after birth, PGE1

Treatment of PDA: Premature infants: All others:

Treatment of PDA: Premature infants: Indomethacin All others: Percutaneous catheter closure or surgical ligation.

A 3-day-old baby girl has a holosystolic murmur along the left lower sternal border. What is the most likely diagnosis?

VSD

A direct connection between both ventricles through a defect in the septum.

VSD

Most common congenital murmur that is holosystolic

VSD

Consists of a continuous low-pitched murmur caused by the collapse of the jugular veins and their subsequent fluttering, and it worsens with inspiration or diastole.

Venous hum

Eisenmenger syndrome: First, there will be a left to right shunting of blood, which will eventually lead to pulmonary hypertension. As the pressures increase in the pulmonary vasculature, more so than the right ventricle, a shunt reversal occurs. This means that deoxygenated blood will be shunted from the right ventricle to the left and out into the systemic circulation. If this occurs, patients will become dyspneic and it may even lead to heart failure if left untreated.

Ventricular Septal Defect

Signs and Symptoms: A holosystolic murmur is heard that does not increase with respiration.

Ventricular Septal Defect

This will usually close without treatment, but surgical repair is done for symptomatic children.

Ventricular Septal Defect Treatment

A 5-week-old infant suffers from dyspnea and fluid overload but not cyanosis. Auscultation reveals a loud, holosystolic murmur at the left sternal border. You suspect a congenital cardiac defect. Echocardiography would most likely show which of the following abnormalities?

Ventricular septal defect in the membranous portion.