heart development
Anomaly.  Clinical Features PaO2 
100%		 Pulm 
flow		 O2? PGE1 mur 
mur?		 image CXR Rx
normal 99% of all hearts are normal (96% in family with one affected child)  >300  normal OK No  No norm do no harm 
ASD 
9% Cong. HD		  usually symptomless, L to R shunt

Females > Males

 300 increase OK No May have one.

split 
S2

 If LV = small, may show increased lung markings. Repair if symptomatic,

Device closure, stitch or patch closure
Coarctation Aorta 

7%Cong.HD

 Abnormal AV, VSD, + PDA. Resistance to aortic blood flow distal to site of narrowing causes a decrease in circulation to lower body. <50 to 
150 
cyanosis if severe		 increase OK Yes Decreased pulses in lower extremities cardiomegaly repair
Ebstein's  Tricuspid valve abnormalities, ASD, small ineffective RV. Patient needs a PDA. There are usually other anomalies <50 
cyanosis		 decrease - Yes - massive RA 
enlargement		 ASD repair, Norward Procedure, BiDirectional Glenn, Fontan
PDA R to L pulmonary hypertensioa <100 decrease Yes no Yes
PDA L to R pulmonary edema increase Yes no
anomaly properties PaO2 in 100% pulm flow give
O2?		 PGE1 murmur imagery cxr Treatment
HLHS

1.5 %

Males>Females 

  Hypoplastic LV, mitral atresia, aortic atresia, hypoplastic aortic arch, large PA. Cyanosis
Patient needs PDA for survival until surgical repair.		 <150 
CHF		 Increase as PDA closes min 
imi 
ze		 Yes Yes CHF as PDA closes Norwood Procedure, BiDirectional Glenn, Fontan, or Transplant
Pulmonary Atresia Progressive cyanosis. Undeveloped pulm valve and obstruction of path to pulmonary artery, Hypoplastic RV, ASD/PFO <50 
CHF		 decrease -- Yes systolic 
murmur 
tricuspid 
regurg		 Hepatomegaly Enlarged heart high mortality
Pulmonary stenosis 
9% Cong. HD		  Risk factor- rubella syndrome <50 decrease - Yes - decrease pulm 
vasc		 -
anomaly clinical features PaO2 in 100% pulm flow give
O2		 PGE1 murmur image CXR Rx
TAPVR Anomalous return of pulm veins-Pulm veins enter R atrium from SVC, IVC, or CS(supracardiac 
cardiac, or infracardiac. Sm LA, Sm LV, PDA, Lg RA, Lg RV 		 <50 increase - Yes Hyperdynamic precordium, loud murmur Increased pulm vasc markings, hepatomegaly requires ASD 
prompt surgery leads to good prognosis
Transposition of the Great Vessels 6% of Congenital HD Cyanosis, Aorta comes off RV and PA comes off LV. Risk factor: maternal insulin dependant diabetes <50 increase Mini-
mal effect		 Yes - egg string ballon septostomy 
switch
anomaly properties PaO2 in 
100%O2		 pulm
flow		 gove O2? PGE1 murmur imagery cxr treatment
Truncus Resembles VSD
Failure of truncus arteriosis to divide 
Risk-maternal thalidomide.		 <150 
CHF		 inc or dec relative to PA status As 
needed		 - - boot 40% mort 
pulm vasc 
obstructive 
disease
Tricuspid Atresia  =
rare		 Cyanosis. Hypo RV, Hypo PA, PFO, PDA, No communication betweeen RA & RV. Need PDA or VSD for pulm blood flow.  R heart Fx leads to CHF  <50 decrease   Yes - - Ballon septostomy, 
Blalock-Taussig  Shunt, BiDirectional Glenn,
Fontan
TOF 

10% Cong. HD 

 Cyanotic. VSD, PS, overriding aorta, RV hypertrophy, PDA. R to L shunting across VSD Volume of right-to-left shunt is directly related to severity of PS. Increased shunting causes "TET" spells 
Risk Factor:fetal alchohol syndrome, maternal trimethadione		 <50 if severe decrease As needed   - Boot-shaped VSD closure 
Enlarge PA, pulm valvotomy 
.VSD -no symptoms to mild 
25% Cong. HD		  either in membranous upper or muscular lower part. Size dependant L-R shunt.
Risk- Fetal alchohol or trimethadione, insulin dependant maternal diabetes.		 300 increase OK No - -  Increased Pulm Vasc markings Stitch, Device, or Patch closure
Anomaly clinical features PaO2 pulm blood O2? PGE1 murmur   CXR Rx