Congenital Heart Disease

Question 1

List some of the most common cardiac malformations

Answer 1

VSD
ASD
PDA
pulmonary stenosis
coarctation of the aorta
others

Question 2

_____ is the most common genetic cause of congenital heart disease

Answer 2

Trisomy 21

Question 3

What cardiac malformation is associated with Turner syndrome?

Answer 3

coarctation of the aorta

Question 4

What cardiac malformation is associated with congenital rubella?

Answer 4

PDA

Question 5

Fetuses have two physiologic ____ to ____ shunts

Answer 5

right to left

Foramen ovale and ductus arteriosus

Question 6

At birth when the baby breathes, pulmonary resistance and thus right sided pressure _______

Answer 6

decreases

Question 7

______ maintain ductus arteriosus patency, ____ causes ductus arteriosus closure

Answer 7

prostaglandins- patency

indomethacin- closure

Question 8

The amount of pulmonary vasculature seen on chest X ray can sometimes be a diagnostic clue. List factors that increase pulmonary vascularity and factors that decrease pulmonary vascularity

Answer 8

Increased: left to right shunting

Decreased: right to left shunting

Question 9

List cyanotic “right to left shunt” cardiac defects present at birth

Answer 9

tetralogy of fallot
truncus arteriosus
tricuspid atresia
total anomalous pulmonary venous connection
transposition of great vessels

Question 10

What is Eisenmenger syndrome?

Answer 10

congenital left to right shunt causes increased pulmonary vascular pressures, eventually the shunt reverses leading to cyanosis later in life

Question 11

What are the four components of the tetralogy of Fallot?

Answer 11

ventricular septal defect
pulmonary stenosis
overriding aorta
right ventricular hypertrophy

Question 12

Describe chest X ray findings in tetralogy of fallot

Answer 12

boot shaped heart- RV hypertrophy displaces the apex superiorly and left laterally

decreased pulmonary vasculature

Question 13

List clinical features of tetralogy of fallot

Answer 13

dyspnea
polycythemia
infective endocarditis
reduced survival

Question 14

Transposition of the great arteries is incompatible with life unless:

Answer 14

VSD or PDA- shunt is present

Question 15

List three congenital defects that cause left to right shunt

Answer 15

ASD
VSD
PDA

Question 16

Reversal of a left to right shunt due to _______ may cause cyanosis late in life

Answer 16

chronic pulmonary overload (pulmonary HTN)

Question 17

VSD is associated with what genetic conditions?

Answer 17

Trisomy 21, 13, 18

Question 18

What is the most common location of a VSD?

Answer 18

Membranous, superior part of the septum

Question 19

Small _____ VSDs may close spontaneously in childhood

Answer 19

muscular

Question 20

List clinical features of VSDs

Answer 20

pulmonary HTN
CHF
pansystolic murmur
Eisenmenger syndrome

Question 21

What is the major risk/ complication with atrial septal defects?

Answer 21

paradoxical embolus

Question 22

Describe the types of ASDs and their relative prevalence

Answer 22

ostium secundum- middle of the septum, 90%
ostium primum- low in the septum, 5%
sinus venosus, 5%

Question 23

A continuous machinery like murmur suggests _______

Answer 23

PDA

Question 24

How can a PDA be closed?

Answer 24

indomethacin

Question 25

Describe associations with coarctation of the aorta

Answer 25

Turner syndrome
bicuspid aortic valve
VSD, ASD
berry aneurysm

Question 26

Describe preductal coarctation of the aorta

Answer 26

presents in infancy

CHF, selective cyanosis of lower extremities, weaker femoral pulses that UE

Question 27

Describe post-ductal coarctation of the aorta

Answer 27

presents in older children and adults
no selective cyanosis
HTN of UE, low BP and weak pulses in LE
notching of ribs due to collaterals
intermittent claudication