04a: Congenital Disease

Question 1

Heart tube forms from fusion of (X), which arise from (Y) layer after gastrulation.

Answer 1

X = endocardial tubes
Y = mesoderm

Question 2

List the four parts of the primitive heart tube (from rostral to caudal).

Answer 2

1. Truncus arteriosus
2. Bulbus cordis
3. Primitive ventricles
4. Primitive atria

Question 3

Primitive ventricle begins contracting on day (X) and folding ends around day (Y).

Answer 3

X = 22
Y = 35

Question 4

Septation of heart: first, the (X) canal is divided when (Y) grow and fuse.

Answer 4

X = atrio-ventricular
Y = endocardial cushions

Question 5

Septation of heart: the (atria/ventricles) are separated first by formation of (X).

Answer 5

Atria;

X = septum primum then septum secundum

Question 6

Foramen ovale is a hole in septum (X).

Answer 6

X = secundum (one-way valve)

Question 7

(X) and (Y) parts of the primitive heart tube form bulbar ridges that fuse in spiral fashion. Which cells give rise to these ridges?

Answer 7

X = bulbus cordis
Y = truncus arteriosus

Neural crest cells

Question 8

Heart embryology: If neural-crest derived (X) do not form, the baby will have (Y) congenital defect.

Answer 8

X = bulbar ridges
Y = persistent truncus arteriosus

Question 9

Heart embryology: If neural-crest derived (X) twist with improper geometry, the mom likely had (X) disease

Answer 9

X = bulbar ridges
Y = DM
(transposition of the great vessels)

Question 10

Fetal circulation: oxygenated blood from placenta gets to fetus via (X), which passes though the fetal liver and drains via the (Y) into the (Z) and then into the heart.

Answer 10

X = umbilical vein
Y = ductus venosus
Z = IVC

Question 11

What’s Eisenmenger’s syndrome?

Answer 11

Long-standing L to R shunt (non-cyanotic) eventually causes pulm vascular R to exceed systemic vascular R, causing R to L shunt and cyanosis

Question 12

Most common atrial septal defect is (superior/central/inferior) with septum (primum/secundum)

Answer 12

Central; secundum

Primum would be inferior

Question 13

T/F: Most atrial septal defects can be closed in the cardiac catheterization lab with a transcatheter device.

Answer 13

False - only ostium secundum (ostium primum or sinus venosus defects closed surgically)

Question 14

Which auscultation findings on physical exam would make you think patient has ASD?

Answer 14

1. ULSB systolic ejection murmur (lots of blood through pulm a)
2. Fixed S2 split
3. Parasternal heave (RVH)

Question 15

(X)% of population has patent foramen ovale, which puts patient at risk for (Y).

Answer 15

X = 20
Y = embolic stroke (DVT can cross to L side of heart)

Question 16

T/F: Most patent foramen ovale defects are closed in the cardiac catheterization lab with a transcatheter device.

Answer 16

False - most not repaired

Question 17

VSD causes (R/L) heart overload. Why?

Answer 17

L; ventricles contract together, so extra blood ejects directly into pulm a (never sits in R heart)

Question 18

List the 4 different types of VSDs and star those that never close spontaneously.

Answer 18

1. Inlet*
2. Outlet*
3. Muscular
4. Membranous

Question 19

T/F: Both ASDs and VSDs could present with systolic ejection murmur at ULSB.

Answer 19

True (increased blood flow across pulmonic valve)

Question 20

T/F: In VSDs, auscultatory findings can give you an idea about the size of the defect.

Answer 20

True (higher pitch murmur, smaller defect)

Question 21

(X) is major risk factor for patent ductus arteriosus defect. How is this typically fixed?

Answer 21

X = premature birth

NSAIDs in the neonatal period (inhibit PGE, which keep it open)

Question 22

Baby born with continuous “machinery-like” murmur. What’s the defect and where are you hearing this murmur?

Answer 22

Patent ductus arteriosus;

over aortic area

Question 23

Coarction of the aorta usually occurs just (proximal/distal) to (X) landmark. Which two conditions/diseases is it usually associated with?

Answer 23

Proximal;
X = ductus arteriosus

Bicuspid aortic valve and Turner’s syndrome