embrylogy and congenital heart disease

Question 1

genetic: complete AV canal

Answer 1

trisomy 21

Question 2

genetics: Holt-oram, ASD

Answer 2

TBX5

Question 3

genetics: conotruncal and arch anomalies

Answer 3

Del 22q11

Question 4

double outlet right ventricle is during or after morphogenesis

Answer 4

during

Question 5

pulm. atresia with small RV during or after morphogenesis

Answer 5

after

Question 6

left ventricle not sufficient to support the systemic circulation leading to mitral/ aortic stenosis or atresia.

—> poor chamber growth due to inadequate flow

Answer 6

hypoplastic left heart syndrome

Question 7

blood vessels arise from

Answer 7

blood islands

Question 8

mesodermal derivatives induced to form

Answer 8

endothelial cells- VEGF

Question 9

normal heart tube loping

Answer 9

rightward loop- D-looped ventricles

Question 10

neural crest cells are important for the formation of

Answer 10

normal AV valves, outflow tract and arch development

Question 11

endocardial cushions develop into

Answer 11

atrioventricular and conotruncal regions to assist with septation

Question 12

atrioventricular cushions form the

Answer 12

crux or center of heart; atrioventricular valves and adjacent portions of atrial and ventricular septum

Question 13

what forms the valve leaflets

Answer 13

mesenchymal tissues

Question 14

muscular cords degenerate into

Answer 14

dense chordae tendineae

Question 15

thick muscular trabeculae persist as

Answer 15

papillary muscles

Question 16

conotruncal endocardial cushions form the

Answer 16

semilunar valves and ouflow tract septum

Question 17

aorticopulmonary septum seperates the

Answer 17

aorta and PA above valves

Question 18

AV canal septum is formed from the

Answer 18

endocardial cushions

Question 19

paired series of aortic arches 1-6 with the pharyngeal arches, lead to paired

Answer 19

dorsal aortae

Question 20

A 9 month year old girl is evaluated in the cardiology clinic for noisy breathing and difficulty swallowing solids. Echocardiography reveals a vascular ring composed of a double-aortic arch.

Which of the following is true concerning her aortic arch development?

A She had only a single dorsal aortic arch in early arch development

B Abnormal neural crest cell migration played an important role

C Incomplete aorticopulmonary septum formation is likely

D One arch did not regress

Answer 20

D One arch did not regress

Question 21

Assuming that valvar pulmonary atresia is compatible with fetal life, which fetal shunt is most critical to that fetus’ survival to term?

A The patent foramen ovale

B The patent ductus arteriosus

C The ductus venosus

Answer 21

A The patent foramen ovale

Question 22

fetal circulation is

Answer 22

parallel

Question 23

failure of delamination

Answer 23

ebstein’s anomaly of the tricuspid vale can lead to non-compaction cardiomyopathy

Question 24

A 5 year old boy with tetralogy of Fallot is having episodic hypercyanotic episodes with walking. He performs one physical maneuver that helps him. What is it?

A Valsalva maneuver

B Lies down

C Squats down

Answer 24

C Squats down

Question 25

failure of normal septation of truncus arteriosus

Answer 25

transposition of the Great Arteries; D-loop TGA