10/30-Congenital Heart Disease

Question 1

What percentage of congenital heart diseases are multifactorial (genetic + environmental?)

Answer 1

90%

Question 2

What is the most common congenital heart defect?

Answer 2

VSD (33%)
ASD, PS, tetralogy, PDA (33%)
All other types 33%

Question 3

T/F- increase in pulmonary flow (ASD) is well tolerated by pulmonary vessels where as an increase in pulmonary BP (VSD) is not well tolerated by pulmonary vessels

Answer 3

true

Question 4

definition, location, and features of atrial septal defect?

Answer 4

• interatrial opening present throughout cardiac cycle
• fossa ovalis
• may be asymptomatic until adulthood, plexiform lesions in <10%

Question 5

definition, location, and features of ventricular septal defect?

Answer 5

• interventricular opening between LV and RV
• at membrane septum (90%)
• -for large, un-operated VSD’s plexiform lesions develop in 100% but none will develop in small lesions

Question 6

T/F- muscular VSD is usually small and spontaneous closure generally occurs via fibrous adhesions so most won’t need surgery

Answer 6

true

Question 7

T/F- membranous VSD is usually a large defect and surgical closure is often required by age 2

Answer 7

true

Question 8

What are the two types of atrioventricular septal defects?

Answer 8

• Partial (primum ADS & cleft MV with MR)

- Complete (AVSD & common AV valve)

Question 9

Atrioventricular septal defect is strongly associated with what syndrome?

Answer 9

down syndrome (40%)

Question 10

When does functional closure normal occur in the ductus arteriousus?

Answer 10

functional (within 12 hrs of birth)

structural (within 3 months of birth)

Question 11

T/F- the L=>R shunt reverses to R=>L in severe pulmonary hypertension

Answer 11

true

Question 12

What is pulmonary stenosis?

Answer 12

PV obstruction due to hypoplasia, dysplasia, or abnormal number of cusps. May be asymptomatic until adulthood.

Question 13

What are secondary cardiac effects of pulmonary stenosis?

Answer 13

pulmonary artery dilatation and RVH and RA dilatation

Question 14

What is aortic stenosis?

Answer 14

AV obstruction due to hypoplasia, dysplasia, or abnormal number of cusps

Question 15

T/F- critical aortic stenosis is usually not symptomatic until later in life

Answer 15

False, severe stenosis symptomatic at birth

Question 16

What are cardiac effects of aortic stenosis?

Answer 16

LV hypertrophy and left atrium dilatation

Question 17

What is coarctation of the aorta?

Answer 17

ridge-like indentation of distal aortic arch, across from ductus arteriosus

Question 18

What are the 2 types of coarctation of the aorta?

Answer 18

with PDA (infantile)
without PDA (adult)

Question 19

T/F- Bicuspid AV is present in 50% and rib notching is often apparent on X-ray in pts with aortic coarctation

Answer 19

true

Question 20

What are the four main characteristics of tetralogy of Fallot?

Answer 20

VSD
Subpulmonary stenosis
Overriding aorta
RV hypertrophy

Question 21

What are the three types of tetralogy of fallot? What are these based on?

Answer 21

pink, classic, PA-VSD

based on severity of sub pulmonary stenosis

Question 22

T/F- Tetralogy of fallot is the most common form of cyanotic CHD

Answer 22

true

Question 23

T/F- In transposition of great arteries, the aorta originates from the RV and the PA from the LV

Answer 23

true

Question 24

What is truncus arteriosus?

Answer 24

origin of aorta and PA from tranquil artery, large VSD present in most

Question 25

Which genetic disease if truncus arteriosus commonly associated with?

Answer 25

DiGeorge syndrome

Question 26

T/F- plexiform lesions generally don’t develop in unoperated truncus arteriosus

Answer 26

False, most will develop plexiform lesions if unoperated

Question 27

What is ebstein anomaly of TV?

Answer 27

adherent septal and posterior leaflets, redundant anterior leaflet, and dilated annulus, with tricuspid regurgitation

Question 28

Secondary effects on the heart of ebstein anomaly?

Answer 28

RV and RA dilatation

Question 29

T/F- Ebstein anomaly of TV is associated with wolf-parkinson-white

Answer 29

true