Pediatric Cardiology

Characteristics of Cyanotic Congenital Heart Defects

Profound cyanosis

Not relieved with hyperoxia test

Dyspnea

2D Echocardiogram makes definitive diagnosis

Tetralogy of Fallot

4 defects:

Ventricular septal defect (VSD)

Pulmonary stenosis

Overriding aorta

Right ventricular hypertrophy

Pulmonary stenosis murmur

Crescendo-decrescendo

Hypoxic (Tet) spells with crying or feeding, put knees to chest

Classic chest x-ray finding is a boot-shaped heart, with an upturned cardiac apex

Cyanotic

Transposition of the great vessels

Cyanosis-profound

No murmurs

Chest x-ray classically an "egg on a string" created by a narrow superior mediastinum

Tricuspid Atresia

The absence of the tricuspid valve results in a hypoplastic right ventricle

Severely cyanotic with single S2

Truncus Arteriosus

A single S2 is due to the single valve

Minimal Cyanosis

Total anomalous pulmonary venous return (TAPVR)

All of the pulmonary veins fail to connect to the left atrium and return abnormally via the right side of the heart

May see supracardiac, infracardiac, cardiac, or mixed drainage

Cyanosis

Characteristics of Acyanotic Heart disease

Lesions result in increase volume load

Shunting of fully oxygenated blood back to the lungs

Pulmonary edema → Tachypnea, chest retractions, nasal flaring, wheezing

Ventricular Septal Defect

Loud, harsh, systolic murmur, usually heard best at the left lower sternal border

No cyanosis

Pulmonary HTN, poor feeding, dyspnea

Atrial Septal defect

Systolic ejection murmur at the left upper sternal border

Eisenmenger syndrome

Uncorrected left to right shunts →

Severe irreversible pulmonary hypertension

Right ventricular failure resulting in right-to-left shunting

Cyanosis, dyspnea with exertion, hepatomegaly, and clubbing of the fingers and toes

Patent Ductus Arteriosus (PDA)

Continuous, machinery-like murmur can be heard at the left infraclavicular area

Coarctation of the Aorta

Narrowing of descending Aorta

Disparity of pulse and BP in arms and legs

Normal BP: higher in leg

In CoA, BP much lower in legs

Check BP in all 4 extremities

Check ipsilateral pulses of radial and femoral

Severe CoA will have absent femoral pulses

Rib notching may be seen in older children (>8 years of age) with large collaterals (commonly from subclavian artery)

Most common heart defects in Down Syndrome

Complete atrioventricular septal defect

Ventricular septal defect

Atrial septal defect

Congenital Rubella Syndrome

In utero infection

Blueberry muffin rash

Deafness, cataracts, and cardiac disease (PDA)

Most common cardiac defect in Turner Syndrome

Coarctation of the aorta

Bicuspid aortic valve leading to aortic stenosis

Hypertension

Prolonged QT

Aortic dissection

Marfan Syndrome

Aortic root dilatation/dissection

Ectopia lentis: lens dislocation

Above average height

Arachnodactyly

Dolichostenomelia (elongated limbs)

Scoliosis is common

Pectus excavatum

Pectus carinatum

Major Jones criteria for diagnosis of rheumatic fever

Migratory arthritis

Carditis and valvulitis

Central nervous system involvement

Erythema marginatum

Subcutaneous nodules- located over extensor surface of elbows, knees, knuckles, and ankles or scalp and spine; firm, nontender

Kawasaki Disease

Acute febrile vasculitis

If untreated, can develop coronary artery abnormalities (aneurysms, dilatation, stenosis...)

Most often proceeded by viral infection

Most younger than 5y.o.

Causes severe vasculitis, mostly medium-sized arteries (coronary)

ECHO: coronary abnormalities, repeat at 2-3 wks, then again at 6-8wks

Treatment of Kawasaki Disease

Treatment should not be delayed

First line:

Aspirin + IVIG within 7-10 days of onset

If IVIG resistance (fever persist > 36hrs after initial dose) then give a 2nd dose

If still not improving- add infliximab or methylprednisone

High risk patients would receive Aspirin/IVIG + methylprednisone

< 6mo, >9yo, Asian race, CRP > 13mg/dL, and Zscore >2 in LAD or right coronary artery