04b: Abnormal Heart Development

Question 1

Atrial septal defects are caused by problems with tissue (formation/breakdown). What are the types of ASDs?

Answer 1

Both;

1. Ostium secundum (75%)
2. Ostium primium (20%)
3. Sinus venosus (5%)

Question 2

Pathophysiologic consequences of ASDs typically begin in (fetal period/childhood/adulthood). List some of these symptoms.

Answer 2

Adulthood;

1. Arrhythmia
2. Paradoxical embolism
3. Pulmonary hypertension
4. RV failure

Question 3

T/F: Patent foramen ovale is a type of ASD.

Answer 3

False

Question 4

In the most common, (X), type of ASD, the size of (Y) is insufficient to cover entire fossa ovale and/or (Z) fails to fully form.

Answer 4

X = ostium secundum
Y = septum primium
Z = septum secundum

Question 5

Ostium secundum defect is typically found in (dorsal/ventral/superior/inferior) portion of atrial septum.

Answer 5

Middle/center

Question 6

The clinical course of patients diagnosed with isolated ASD ostium secundum defect is often how severe?

Answer 6

Benign; may be associated with few functional limitations

Question 7

A larger, more severe ostium secundum defect may lead to (X) due to persistent (Y).

Answer 7

X = RA volume overload;
Y = L to R shunting

Question 8

ASD ostium primum defect is (more/less) common and (more/less) severe than ostium secundum defect.

Answer 8

Less common; more severe

Question 9

ASD ostium primum defect results in (L/R) to (L/R) shunting between (X) chambers due to defective septation between which surfaces of (Y) and (Z)?

Answer 9

L to R;
X = atria
Y = inferior margin of septum primum
Z = atrial surface of endocardial cushion

Question 10

Severe ASD ostium primum defect will manifest as (X) in which stage of life?

Answer 10

X = heart failure

First few months

Question 11

ASD sinus venosus defect is found in (upper/middle/lower) portion of (X) septum, near (Y) vessel opening.

Answer 11

Upper;
X = atrial
Y = SVC

Question 12

T/F: ASD sinus venosus defect has the most poor prognosis of the ASDs.

Answer 12

False - excellent prognosis with surgical repair

Question 13

ASD sinus venosus defect: the (R/L) (X) vessel may faultily drain into which chamber?

Answer 13

R;
X = pulmonary vein
RA

Question 14

T/F: Presence of patent foramen ovale doesn’t always imply active interatrial communication.

Answer 14

True

Question 15

List some conditions in which patent foramen ovale unsealed flap may open.

Answer 15

Higher R side pressure:

Sustained pulmonary HT, sneezing, coughing

Question 16

Most common form of congenital cardiac anomaly is (X), which is usually associated with other cardiac anomalies.

Answer 16

X = Ventricular Septal Defect

Question 17

Most VSDs are related to problems with (formation/breakdown) of (muscular/membranous) part of IV septum.

Answer 17

Incomplete or only partial formation;

Membranous

Question 18

Most VSDs are typically the size of (X) orifice.

Answer 18

X = aortic valve

Question 19

Large and severe VSDs create (X) shunt, leading to (Y) and (Z) symptoms.

Answer 19

X = L to R
Y = RV hypertrophy
Z = pulmonary hypertension

Question 20

In persistent truncus arteriosus defect, there’s essentially a common trunk of origin for which vessels?

Answer 20

1. Aorta
2. Pulmonary arteries
3. Coronary arteries

Question 21

Persistent truncus arteriosus defect results from incomplete partitioning of (X) by (Y).

Answer 21

X = truncus arteriosus
Y = spiral septum

Question 22

T/F: Persistent truncus arteriosus defect always accompanied by IV septal defect.

Answer 22

True

Question 23

A Persistent truncus arteriosus receives blood from which chamber(s)?

Answer 23

Both ventricles

Question 24

A Persistent truncus arteriosus defect presents with (increased/decreased) pulmonary blood flow, pulmonary (hypo/hyper)-tension, and (X).

Answer 24

Increase;
hypertension;
X = recurrent respiratory infections

Question 25

ASD ostium secundum caused by (incomplete formation/breakdown) of:

Answer 25

1. Incomplete formation of septum secundum OR | 2. Excessive cell death/resorption of septum primum

Question 26

(X) defect may result in anomalous pulmonary venous drainage into RA or SVC.

Answer 26

X = ASD (sinus venosus)

Question 27

An incomplete seal of foramen ovale occurs in about (X)% of adults.

Answer 27

X = 25

Question 28

List the categories of VSDs, depending on various sizes/locations.

Answer 28

1. Small hole in membranous septum 2. Large hole involving more than membranous septum 3. Defect only in muscular portion 4. Complete absence of muscular septum

Question 29

A persistence truncus arteriosus is ALWAYS associated with which two features?

Answer 29

1. VSD | 2. Mixing of LV and RV blood

Question 30

List the four changes that define tetralogy of Fallot.

Answer 30

1. Pulmonary stenosis 2. VSD 3. Overriding aorta 4. RV hypertrophy

Question 31

(X) is the most common R to L congenital heart disease.

Answer 31

X = tetralogy of Fallot

Question 32

The (ASD/VSD) associated with tetralogy of Fallot is incomplete closure of (X), affecting (Y) septum as well as (Z).

Answer 32

VSD; X = membranous septum Y = muscular septum Z = endocardial cushions

Question 33

T/F: In tetralogy of Fallot, the development of spiral septum is abnormal.

Answer 33

True

Question 34

In tetralogy of Fallot, there is a (R/L) displacement of (X) vessel.

Answer 34

R; | X = aorta

Question 35

Clinical consequences of tetralogy of Fallot depend primarily on severity of:

Answer 35

Pulmonary stenosis

Question 36

A (R/L) to (R/L) shunt produces cyanosis.

Answer 36

R to L

Question 37

Transposition of great vessels: aorta is (anterior/posterior) and (R/L) of pulmonary artery and arises from (X) chamber.

Answer 37

Anterior; R; X = RV

Question 38

(X) congenital heart defect, if left untreated, is the leading cause of death in neonates and infants.

Answer 38

X = transposition of great vessels

Question 39

(X) heart defect is actually incompatible with life in absence of accompanying (Y) septal defects.

Answer 39

``` X = transposition of great vessels; Y = interarterial, interventricular, or patent ductus arteriosus ```

Question 40

List the types of congenital aortic stenosis, which are present (alone/in combination). Star the most common type.

Answer 40

1. Valvular* 2. Subvalvular 3. Supravalvular Either

Question 41

In valvular aortic stenosis, the valve cusps may have which problematic characteristics?

Answer 41

May be: hypoplastic, dysplastic or bicuspid

Question 42

Valvular aortic stenosis is due to abnormal development of which key embryological structures?

Answer 42

Endocardial cushions in outflow tract

Question 43

T/F: Valvular aortic stenosis may be asymptomatic in kids.

Answer 43

True

Question 44

Subvalvular aortic stenosis is due to abnormal development of which key embryological structures?

Answer 44

Band of subvalvular fibroelastic tissue or muscular ridge (below valve)

Question 45

Subvalvular aortic stenosis results in (X) of the aortic cusps.

Answer 45

X = thickening or immobility

Question 46

What would the surgical intervention to correct subvalvular aortic stenosis involve?

Answer 46

Removal of fibrous ring (immediately below aortic valve)

Question 47

Supravalvular aortic stenosis is (more/less) common than subvalvular stenosis. Its defining feature is (X).

Answer 47

Less (rarest); | X = focal narrowing above aortic valve (and involving ascending aorta)

Question 48

What would the surgical intervention to correct supravalvular aortic stenosis involve?

Answer 48

Enlargement of narrowed region

Question 49

Atrial septal defects occur in various locations and are described based on relationships to the (X) and (Y) septum.

Answer 49

``` X = atria Y = AV ```