Congenital Heart Disease

Question 1

5 Ts

Answer 1

Tetrology
Transposition
Truncus
Tricuspid atresia
TAPVR 

Can also be Ebstein, HRHS, HLHS

Question 2

Eisenmerger syndrome

Answer 2

Noaways late dx ASD in adult

Question 3

AV canal
Coarctation
Truncus
Pulmonis stenosis

Answer 3

Trisomy 21
Monosomy X
DiGeroge
Noonan (12q22)

Question 4

Supravalvular AS
PDA
TGA

Answer 4

William syndrome (chr 7, elastin)
Congenital rubella syndrome
Infant of diabetic mother

Question 5

Comps of R-L shunt

Answer 5

Cyanotic

Dec pulmonary blood flow

Grwoth retard, polycyth, clubbing, cerebral hypoxia, paradoxic embolism

Question 6

TOF

Answer 6

Sx depend on degree of RVOT obstruction

Mild - similar to isolate VSD

Mod to severe - R-L in 6 mo…classic

Tet spells due to RVOT spasm

Question 7

TGA

Answer 7

Requires shunt for survival

VSD stbale
PFO unstable and need a baloon

if unrepaired, RVH develops

Arterial switch is main way to repair

Question 8

COngenitally corrected TGA

Answer 8

Hypertrophy and late decompensation of the RV

COmplete repair requires both atrial and arterial switch

Question 9

Truncus arteriosus

Answer 9

Single great artery with underlying VSD

Early cyanosis and inc pulmonary blood flow

DiGeorge/velocardiafcial syndrome - Retinoids

Question 10

Tricuspid atresia

Answer 10

Large MV orifice

Associated RV hypoplasia

Need ASD/PFO (right to left) for outlet from RA

Need VSD for outflow to PA

Question 11

TAPVR

Answer 11

All 4 pulmooanry veins fail to drain into LA
Need ASD/PFO for pulmonary venous blood to enter the LA

Cyanosis due to large R-L shunt via ASD

Secondary RA and RV hypertrophy with dilated PA and LA hypoplasia

Question 12

Left to right shunt

Answer 12

inc pulmonary blood flow so no dec in O2 sat

Question 13

Comps of L-R shunt

Answer 13

Eisenmerger - overciculation of lungs leads to pulm HTN…PVR>SVR so becomes R to L shunt and get cyanosis

Question 14

ASD

Answer 14

Flow murmur

Most are secundum

Get lung trnasplant and close ASD

Question 15

VSD

Answer 15

2 types - most common is perimembranous

Question 16

AVSD

Answer 16

Associated with down syndrome

Failure of endocardial cushions to fuse

Question 17

PDA

Answer 17

Continuous harsh murmuor

Indomethacin is Rx tx

Question 18

Coarctation

Answer 18

Narrowing of aorta

In adult - tubular hypoplasia prox to PDA

Turner syndrome

With PDA - surgery as neonate

Without PDA - asx…UE HTN as adult and weak LE pulses

Question 19

Congenital aortic stenosis and atresia in neonate

Answer 19

Neonatal critical aortic valve stenosis - small or dysplastic cusp…if severe, associated with hypoplastic left heart syndrome

Subvalvular stenosis - rung or collar

Question 20

Williams sx

Answer 20

Supravavlular aortic stneosis

Elfin face

Thickening and constriction of aortic wall

Elastin gene of chr 7

Question 21

Noonan syndrome

Answer 21

Short webbed neck with ear malformations

Pulm stenosis 
Hypertrophic CM (rasopathy), and mitral valve prolaspe