Congenital Heart Disease

Shunt L-R

ASD

Often asymptomatic; spontaneous closure if <8mm

CHF, PAH in adult life

ECG: RAD, RVH, RBBB

CXR: increased pulmonary vasculature

Mx: surgery/catether closure between 2-5 yrs

VSD

small

most common CHD; asymptomatic ==> spontaneous closure

Mod-Large

2ary PAH; CHF by 2 months

ECG: LAD, LVH, RVH

CXR: CHF features

Mx: CHF management; surgical closure by 1 year

PDA

asymptomatic; may have apneic/bradycardic spells; poor feeding

tachycardia, bounding pulses, machinery murmur, hyperactive precordium

ECG: LAH, LVH, BVH

CXR: mildly enlarged heart, increased pulmonary vasculature, proeminent pulmonary artery

Echocardiogram establishes diagnosis

Mx: surgical/catheter closure if persistent after 3 months

AVSD

common in Down´s syndrome

Mx: surgery by 6 months to prevent PAH

Obstructive

pallor, decreased UO, cool extremities, poor pulses

CoAo

50% with bicuspid aortic valve; 35% Turner syndrome

often asymptomatic; higher SBP and stronger pulses in upper limbs

ECG: RVH in early infance; LVH later in childhood

Complication: HTN

Mx:

prostaglandin (keep ductus arteriosus open) for stabilization;

surgical/balloon correction

Aortic Stenosis

Dyspnea + Syncope + Chest pain; ejection click

Mx: surgical correction; exercise restriction

Pulmonic Stenosis

usually part of other lesions

asymptomatic to CHF

ECG: RVH

Mx: surgical repair

0,8% of births

Risk Factors

Prematurity ==> PDA

Down's Syndrome ==> AVSD

Marfan / Ehler-Danlos ==> Mitral prolapse, aortic abnormalities

Turner ==> Aortic Coarctation, bicuspid aortic valve

DM ==> TGA

SLE ==> complete heart block

TORCH (Rubella)

Alcohol ==> ASD, VSD

Sibling with CHD

Medications ==> Phenytoin, retinoic acid, valproate

Innocent

do not warrant investigation!!!

asymptomatic

systolic ejection

</= 3/6 +

no extra sounds/clicks

varies with position

Pathologic

Symptomatic

Dyatolic ,pansystolic or continuous

>3/6 +

Unchanged with position

R-L Shunt

TOF

VSD, RVOTO, overriding aorta, RVH

may have L-R shunt initially; progressive RVOTO causes R-L shunt

hypoxic spells caused by exertion

peak 2-4 months

Mx: O2, knee-chest, morphine, propranolol

ECG: RAD, RVH

CXR: "boot-shaped" heart; decreased pulmonary flow

Mx: surgery

Ebstein´s anomaly

defect of the tricuspid valve ("atrialization of RV")

Lithium / benzodiazepine use in 1st trimester

massively enlarged RA, patent foramen ovale ==> R-L shunt

Mx: surgery

Others

Transposition of the Great Arteries

Progressive cyanosis unresponsive to oxygen ==> less dramatic if VSD present

ECG: RAD, RVH

CXR: "egg on a string" (narrow mediastinum)

Mx:

Prostaglandin

Surgery within 2 weeks

Tricuspid Atresia

Totally Anomalous Pulmonary Venous Return

Hypoplastic Left Ventricle

Truncus Arteriosus