Congenital heart disease

Question 1

holosystolic murmur (HSM) at the lower left sternal border (LSB) that is unchanged with standing or the Valsalva maneuver

Answer 1

VSD

Question 2

Standing and Valsalva

Answer 2

decreased venous return, increase HCM murmur

Question 3

easy bruising,” hypertelorism, bifid uvula, cleft palate, pectus excavatum, and aortic root aneurysm

Answer 3

Loeys-Dietz syndrome (LDS), aortic root > 4.2 by echo or >4.4 by CT/MR: intervene. TGFBR1 gene.

Question 4

Asymptomatic aortic repair

Answer 4

4.5 for Marfan, >5.5 for bicuspid

Question 5

vascular Ehlers-Danlos syndrome (EDS) vs Marfan

Answer 5

thin, translucent skin with easy bruising, mitral valve prolapse and POTS. Mutation COL3A1

Question 6

headache, dizziness, slow mentation, a sense of dissociation, tinnitus, paresthesias, myalgias, restless legs, and bleeding or stroke.

Answer 6

hyperviscosity syndrome. Can occur in setting of eisenmenger after dehydration. diagnose with hgb level, treat with fluids, iron if needed->Erythropheresis

Question 7

eisenmenger management

Answer 7

obtain yearly hematocrit, uric acid, transferrin, iron, and ferritin. Iron deficiency is common and should be treated.

Question 8

refractory HTN+ diastolic forward flow in the abdominal aorta

Answer 8

coarct. Intervene if: peak-to-peak gradient >20 mm Hg, clinically significant collateral flow, systemic HTN attributable to CoA, or heart failure attributable to CoA. Treat with stent placement.

Question 9

Complication with greatest risk during Marfan pregnancy

Answer 9

aortic dissection. If > 4cm, replace before preg. Preg CI if >4.5

Question 10

Post coarct repair surveillance

Answer 10

q3-5 years with CT or MR

Question 11

TOF with severe PR. Intervene when

Answer 11

Symptomatic.
Asympt + RV/LV systolic dysfunction OR severe RV dilation OR RVOT obstruction

Question 12

cyanosis and clubbing of the toes and not the fingers

Answer 12

PDA. continuous “machinery”-type murmur-> becomes diastolic murmur with eisenmenger

Question 13

Secundum ASD with L-R shunting. Close if:

Answer 13

PVR < 1/3 SVR,
PASP <50% systemic,
right heart enlargment, Qp/Qs> 1.5. TEE, then RHC for workup

Question 14

Ischemic eval for anomalous coronaries

Answer 14

has to be exercise as it dilates aorta.

Question 15

restrictive asymptomatic VSD

Answer 15

F/u echo in 2 years

Question 16

Close small VSDs if :

Answer 16

symptoms OR
left heart enlargement with 1.5:1 shunt OR
pulmonary hypertension (as long as [PA] systolic pressure is <50% the systemic and the pulmonary vascular resistance is < 1/3 SVR OR
endocarditis

Question 17

turbulent flow in the mid RV

Answer 17

double chambered RV due to muscle bundles that separate a proximal high-pressure chamber from the distal, lower-pressure one

Question 18

ASD closure CI

Answer 18

Eisenmenger

Question 18

ASD workup

Answer 18

Define anatomy first with TEE/MR. Then RHC for physiology specifics

Question 19

Positional hypoxemia, platypnea-orthodeoxia syndrome, better when laying down.

Answer 19

PFO

Question 20

Holt-Oram syndrome. DiGeorge syndrome. Williams syndrome.

Answer 20

secundum ASD.
TOF.
supra-aortic stenosis and hypercalcemia.

Question 21

Anomalous coronaries
left from right.
right from left.

Answer 21

automatic surgery.
surgery if there is ischemia or arrhythmias.

Question 22

ALCAPA

Answer 22

right-to-left shunting from the coronary steal into the pulmonary artery, and the LCA would be markedly dilated over time due to chronic shunting.

Question 23

Trisomy 21

Answer 23

AV canal defect: AV valves in same plane, primum ASD, VSD

Question 24

18-60 years of age with a nonlacunar stroke and PFO

Answer 24

(MRI) of the brain, MRI or CT of intra- and extracranial vessels, venous doppler, event monitor, echocardiography. Then, consider high risk (atrial septal aneurysm or large (>20 bubbles) right-to-left shunt: if so, close.

Question 25

Marfan aortic surveillance

Answer 25

annual

Question 26

Right heart enlargement without ASD on TTE.

Answer 26

Do not repeat TTE with saline. TEE/CMR

Question 27

ebstein surgical repair

Answer 27

decline in exercise capacity and clinical heart failure.

Question 28

TOF ICD

Answer 28

LV systolic or diastolic dysfunction, NSVT, QRS duration ≥180 msec, extensive RV scarring, or inducible sustained ventricular tachycardia (VT

Question 29

Bicuspid aorta surveillance

Answer 29

If > 4.5 cm, annual surveillance

Question 30

VSD closure CI

Answer 30

severe PH.
*Pregnancy is CI in eisenmenger. *

Question 31

systolic ejection click that occurs early in systole, left second interspace and the apex, does not change with any maneuvers

Answer 31

bicuspid valve