Congenital Heart Disease- Gupta Flashcards

1
Q

When do congenital defects occur during pregnancy?

A

4-9th weeks ususally

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2
Q

Causes of congenital defects (maternal factors)?

A
  • infection, rubella
  • alcohol
  • radiation
  • teratogenic drugs
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3
Q

What common birth defect has a strong association with congenital heart malformation?

A

Down’s syndrome

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4
Q

What heart malformations are common with Down’s syndrome?

A
  • AV septal defect (aka Endocardial cushion defect)
  • Ventricular septal defect
  • Persistent ductus arteriosus
  • TOF
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5
Q

What are some left to right shunts?

A
  • ASD (and patent foramen ovale)
  • VSD (most common)
  • Patent ductus arteriosus
  • AV septal defect
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6
Q

What is VSD associated with?

A

pansystolic murmur

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7
Q

What is a patent ductus arteriosus associated with?

A

rubella, continuous “machine” murmur

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8
Q

T or F. Cyanosis is a symptom of left to right shunts

A

F. It’s not.

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9
Q

What is Eisenmenger syndrome caused by?

A

prolonged left-to-right shunting with volume and pressure overloads eventually causing pulmonary HTN and secondary right-sides pressure that exceed those on the left, leading to a reversal of the shunt and the development of cyanosis

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10
Q

What is an ASD?

A

an abnormal fixed opening in the atrial septum that allows unrestricted blood flow between the atrial chambers

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11
Q

The majority (90%) of ASDs are so-called ____ ____ defects.

A

ostium secundum

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12
Q

What is ostium secundum ASD?

A

growth of the septum scandium is insufficient to occlude the second ostium

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13
Q

Significance of a foramen ovale?

A

paradoxical embolism

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14
Q

T or F. Most VSDs are in the muscular septum

A

F. 90% are in the membranous septum, and the remaining 10% are in the muscular septum

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15
Q

What is the ductus arteriosus?

A

pulmonary artery and aorta connection allowing blood entering the pulmonic circulation to be diverted to the body (lungs are high resistance, systemic is relatively low)

soon after birth, the ducts normally closes

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16
Q

What does a patent ductus arteriosus result in?

A

if the ductus remains open, there is a left-to-right shunt potentiating congestive failure

17
Q

What are some right-to-left shunts?

A
  • Tetrology of Fallot (TOF)
  • Transposition of the great arteries
  • Persistent truncus arteriosus

“blue babies”

18
Q

What are the main characterizations of TOF?

A
  • pulmonary stenosis
  • overriding aorta (aorta can get blood from either ventricle)
  • right ventricular hypertrophy
  • ventricular septal defect
19
Q

How does the heart appear with TOF?

A

boot shaped from right ventricular hypertrophy

20
Q

When would a patent ductus arteriosus be helpful?

A

transposition of the great arteries

21
Q

What is a persistent truncus arteriosis?

A

primitive trunks does not divide into the pulmonary artery and aorta properly resulting in a single large arterial trunk that overlies a large ventricular septal defect

accounts for 1-2%of congenital heart anomalies

22
Q

What is a common cause of persistent truncus arteriosis?

A

35% of patients have 22q11 deletion syndrome, which includes DiGeorge syndrome and velocardialfacial syndromes

23
Q

What are the two forms of aortic coarctation?

A

infantile form-preductal (before DA)

adult form- postductal

24
Q

Aortic coarctation is more common in men or women?

A

Men

25
Q

Aortic coarctation in women is associated with what?

A

Turner’s syndrome

26
Q

50% of cases also have what?

A

bicuspid aortic valves

27
Q

Impact of preductal aortic coarctation?

A
  • typically has a PDA (unoxygenated blood to the aorta)
  • dilated pulmonary trunk
  • right ventricular hypertrophy
  • cyanosis of lower half of the body
28
Q

Impact of postductal aortic coarctation?

A
  • no PDA
  • constriction at the ligament arteriosum
  • aortic arch and branch dilation
  • left ventricle hypertrophy
29
Q

Is high BP is the upper body and low BP in the lower body associated with pre- or postductal AC?

A

postductal

30
Q

What is another effect of post ductal AC?

A

increased pressure will increase renal pressure leading rib notching (erosion of the inferior margins of the ribs)

aka Roesler sign

31
Q

Is rib notching always seen?

A

only in long standing cases, and therefore not seen in infancy (unusual in patients under 5 y/o)

32
Q

Aortic coarctation can lead to what?

A

atherosclerosis

33
Q

Association with Down syndrome?

A

septal defect

34
Q

Association with rubella?

A

PDA

35
Q

Association with Turner syndrome?

A

coarctation of the aorta

36
Q

Association with 22q11.2DS?

A

TOF

37
Q

Association with fetal alcohol syndrome?

A

VSD/ASD

38
Q

Association with maternal DM types I and II?

A

transposition of the great vessels