04b: Abnormal Heart Development Flashcards

1
Q

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

A

Both;

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

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

A

Adulthood;

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

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

A

False

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4
Q

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.

A
X = ostium secundum
Y = septum primium
Z = septum secundum
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5
Q

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

A

Middle/center

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6
Q

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

A

Benign; may be associated with few functional limitations

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7
Q

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

A
X = RA volume overload;
Y = L to R shunting
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8
Q

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

A

Less common; more severe

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9
Q

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)?

A

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

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10
Q

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

A

X = heart failure

First few months

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11
Q

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

A

Upper;
X = atrial
Y = SVC

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12
Q

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

A

False - excellent prognosis with surgical repair

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13
Q

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

A

R;
X = pulmonary vein
RA

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14
Q

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

A

True

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15
Q

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

A

Higher R side pressure:

Sustained pulmonary HT, sneezing, coughing

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16
Q

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

A

X = Ventricular Septal Defect

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17
Q

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

A

Incomplete or only partial formation;

Membranous

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18
Q

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

A

X = aortic valve

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19
Q

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

A
X = L to R
Y = RV hypertrophy
Z = pulmonary hypertension
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20
Q

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

A
  1. Aorta
  2. Pulmonary arteries
  3. Coronary arteries
21
Q

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

A
X = truncus arteriosus
Y = spiral septum
22
Q

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

A

True

23
Q

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

A

Both ventricles

24
Q

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

A

Increase;
hypertension;
X = recurrent respiratory infections

25
Q

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

A
  1. Incomplete formation of septum secundum OR

2. Excessive cell death/resorption of septum primum

26
Q

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

A

X = ASD (sinus venosus)

27
Q

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

A

X = 25

28
Q

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

A
  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
29
Q

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

A
  1. VSD

2. Mixing of LV and RV blood

30
Q

List the four changes that define tetralogy of Fallot.

A
  1. Pulmonary stenosis
  2. VSD
  3. Overriding aorta
  4. RV hypertrophy
31
Q

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

A

X = tetralogy of Fallot

32
Q

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

A

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

33
Q

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

A

True

34
Q

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

A

R;

X = aorta

35
Q

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

A

Pulmonary stenosis

36
Q

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

A

R to L

37
Q

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

A

Anterior;
R;
X = RV

38
Q

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

A

X = transposition of great vessels

39
Q

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

A
X = transposition of great vessels;
Y = interarterial, interventricular, or patent ductus arteriosus
40
Q

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

A
  1. Valvular*
  2. Subvalvular
  3. Supravalvular

Either

41
Q

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

A

May be: hypoplastic, dysplastic or bicuspid

42
Q

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

A

Endocardial cushions in outflow tract

43
Q

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

A

True

44
Q

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

A

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

45
Q

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

A

X = thickening or immobility

46
Q

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

A

Removal of fibrous ring (immediately below aortic valve)

47
Q

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

A

Less (rarest);

X = focal narrowing above aortic valve (and involving ascending aorta)

48
Q

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

A

Enlargement of narrowed region

49
Q

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

A
X = atria
Y = AV