Clinical Congenital Heart Disease

Question 1

Embrylogy that is relevant

Answer 1

L-loop - left ventircle ends up on the right

Four cushions develop at AV junction which will become the AV valves

atrial septum made of septum secundum and septum primum

Primum makes up the valve of the F O

Question 2

Down syndrome
Turner syndome
Noon syndrome
Alagille syndrome

Answer 2

AV septal defect
Coartation/BAV
Pulm stenosis/HCM
Branch PA stensois

Question 3

Neonatal transition

Answer 3

Neonate - large PDA with high PVR and eqaul Ao and PA pressure

First few weeks - PDA closes, PVR decreases, PA pressure decreases

Thereafter - DA closed…PVR much lower than SVR…PA pressure much lower than Ao

Question 4

CHF vs cyanosis

Answer 4

CHF - pulmomary ovecirculation (L to R) and/or impaired perfusion…shunt lesions and/or pump failure

Cyanosis - right to left shunting leading to hypoxemia

Question 5

Acyanotic hd

Answer 5

Obstructive without shunt - aortic and pulmonic stneosis…coarctation

Shunt without obstruction - ASD/VSD, PDA, AV canal

Question 6

Cyanotic HD

Answer 6

SHunt with obstruction - VSD with pulm stneosis, tetralogy

Transporition

Mxing lesions

Question 7

ASD

Answer 7

Rarely sx

Inc flow but normal RA pressure

Progressive RH failure and enlargement

Arrhytmia

Indication to locse is a significant L to R shunt with right heart enlargement

Question 8

VSD

Answer 8

L to R shunt

Irreversible pulm vascular dz

Progressive LH enlargemnt

Close if signifncant shunt with progressive heart chamber enlargement

Diuretics, +/- digoxin, +/- afterload reduction

Question 9

Eisenmenger syndrome

Answer 9

From VSD

Longstanding L to R hsunt leads to R to left shunt due to high pulmonary vascular resistance

Cyanosis

Question 10

Eisenmerger signs and sx

Answer 10

Cyanosis
Polycythemia
Hyperviscosity

Question 11

AV canal

Answer 11

Basic on large chamber for everything

Technically a left to right shunt

Question 12

PDA

Answer 12

From aorta into pulmonary trunk

Comps include CHF, arrhythmias, pulm HTN

Tx with diuretics +/- afterload reducction

Close if significant shunt with chamber nelargement…if no murmur or enlargement, do NOT close

Question 13

Pulm stenosis

Answer 13

Well tolerated in generall
Intervene if high pressure in RV (even without sx) or if sx

Diuretics +/- inotropes

Balloon vavuloplasty is preferred option

Question 14

Aortic stenosis

Answer 14

Balloon valvuloplasty is preferred when severe but may need surgery eventually

Question 15

Coarctation

Answer 15

Right arm HTN

Management - BP control…surgery for young, transcatheter for older and children

LT comps - HTN, anyeruyms, aortic valve dz

Question 16

Def of cyanosis

Answer 16

5 gm of reudced Hb per dL cap blood

Normally right to left shunt

Distinuish from hypoxia

Question 17

5 Ts

Answer 17

Transposition
Tetralolgy of fallot
Truncus arteriosus
Tricuspid atresia
Total anomalous pulmonary venous return

Question 18

Comps of cyanosis

Answer 18

CVA under 2
Brain abscess over 2

Polycythemia and relative anemia

Question 19

Taterology

Answer 19

VSD
Aoritc override
Pulm stenosis
Right ventricular hypertrophy

Present as infant with murmur and cyaosis

Question 20

Tet spells

Answer 20

Hypercyanotic spells due to inc in right to left shunt triggered by cyring

Tx with knee to chest, volume, oxygen, beta blockers

Question 21

BT shunt

Answer 21

Basically a created PDA from subclavian to pulmonary artery