Pediatric Medicine: Exam 1 lec 5

what is the most common location for coarctation of the aorta?

juxta-ductal (occurring opposite the entry of the ductus arteriosus)

acyanotic congenital heart diseases are ________________ to _______________ shunts cyanotic congenital heart diseases are ________________ to _______________ shunts

left to right right to left

how do you treat RF?

long-term penicillin prophylaxis

what murmur is most commonly associated with RF?

mitral stenosis

what are the most common cyanotic congenital heart defecets?

the 5 T's: 1. Truncus arteriosus 2. Transposition of the great arteries 3. Tricuspid atresia 4. Tetralogy of Fallot 5. Total Anomalous Pulmonary Venous Return

what are the 4 problems of Tetralogy of Fallot (TOF)?

1. "Malalignment" type of ventricular septal defect (VSD) 2. The aortic root overrides the ventricular septum 3. Pulmonic stenosis 4. Right ventricular hypertrophy

what is characteristic of the subacute phase of KD?

-Desquamation of the skin -Reactive thrombocytosis -Development of coronary artery aneurysms

what is characteristic of the acute phase of KD?

-High, hectic fever, followed by: -Conjunctival erythema -Mucosal changes -Cervical lymphadenopathy -Swelling of the hands and feet -Elevated inflammatory parameters

characteristics of innocent murmurs?

-the quality of the sound -lack of significant radiation -a significant alteration in the intensity of the murmur with positional changes -most important, the cardiovascular history and examination are otherwise normal

how do you treat transposition of the great arteries ?

1. Prostaglandin E1 to keep ductus arteriosus open (survival is not possible without a ASD, VSD or PDA) 2. Arterial switch surgery

grade I or II systolic ejection murmur and fixed split S2 ...

ASD

what are the 2 diastolic murmurs?

Aortic regurgitation/insufficiency Mitral stenosis

what is the initial treatment of KD?

Aspirin at anti-inflammatory doses (80-100 mg/kg/day divided every 6 hours) in the acute phase

what is the main treatment of KD?

IV Ig *shown to reduce the incidence of coronary artery aneurysms from 20-25% to <5%

red blood shot eyes, red cracked lips and red tongue, swollen hands and feet, high fever, and whole body rash are hallmarks of...

Kawasaki Disease

what is the second most common vasculitis of childhood?

Kawasaki Disease (KD)

what is the most common abnormal continuous murmur?

PDA *can also be heard with coarctation of the aorta when collateral vessels are present

continuous machine-like murmur heard at the upper left sternal border...

PDA *systolic-diastolic murmur (continuous)

when you see erythema marginatum suspect ...

Rheumatic fever

when you see a child that has a history of squatting while playing and a boot shaped heart on CXR suspect ...

TOF

high-pitched and holosystolic murmur and is best heard at the left sternal border or apex ...

VSD *small VSDs may be louder than large ones due to the higher flow velocity across smaller defects

what is the most common congenital cardiac malformation?

VSD (42%)

what is a patent ductus arteriosus (PDA)?

a persistent communication between the aorta & pulmonary artery that lasts beyond the immediate postpartum period *more common in infants born before 28 weeks GA or those weighing <1500 g at birth *more common in females

What is Kawasaki disease?

a vasculitis that typically affects children under the age of seven *characterized by multisystem involvement and inflammation of small- to medium-sized arteries with resulting aneurysm formation *runs a triphasic course: acute, subacute, and convalescent

what is the most important manifestation of KD?

aneurysmal involvement of the coronary arteries

what is the most common major manifestation of RF?

arthritis *it usually involves the large joints and is migratory. *arthralgia cannot be used as a minor manifestation if arthritis is used as a major manifestation.

when does the fetal heart develop?

by 6 weeks gestation

how do you diagnose Rheumatic fever?

clinical and laboratory findings of the revised Jones criteria -polyarthritis -carditis -chorea -erythema marginatum -subcutaneous nodules

what congenital heart defect should be suspected in an adult patient with unexplained hypertension, heart failure, exercise intolerance, or underdeveloped lower extremities?

coarctation of aorta

what is the next best step if you're concerned about a murmur?

echo

what is the preferred method to diagnose VSD?

echo *will give you information about size/location, relationship to the pulmonary, aortic, and tricuspid valves, presence of obstruction to outflow of the left or right ventricles and/or prolapse of the aortic valve, right ventricular pressure and function, and evaluation for additional congenital heart disease

what should you suspect in infants that exhibit poor growth, with weight being more significantly affected than height and head circumference, fatigue or diaphoresis with feeding?

heart failure

what is the most common cause of death from cardiac defect in the first month of life?

hypoplastic left heart syndrome *occurs when there is failure of development of the mitral or aortic valve *a small left ventricle that is unable to support normal systemic circulation is a central finding

what is the result of the absence of the tricuspid valve in tricuspid atresia?

hypoplastic right ventricle

what is characteristic of the convalescent phase of KD?

occurs 6-8 weeks into the illness, is characterized by the resolution of symptoms and the development of dystrophic nail changes

what is the initial management to maintain or reopen the ductus arteriosus? what is the initial management to induce ductal closure?

prostaglandin E1 Indomethacin or ibuprofen (NSAIDs)

what congenital heart defect do the pulmonary veins fail to connect to the left atrium?

total anomalous pulmonary venous return

what is the most common cyanotic lesion to present in the newborn period?

transposition of the great arteries

what cyanotic congenital heart defect is associated with DiGeorge syndrome and diabetic mothers?

truncus arteriosus