Pathoma: Congenital Heart Defects Flashcards

1
Q

What is the most common congenital heart defect?

What causes it?

A

VSD

fetal alcohol syndrome

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2
Q

VSD: A left to right or right to left shunt? Why?

A

left to right bc the pressure in the systemic circuit is greater than going back to the RV, so it does

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3
Q

large VSD –> increase in blood flow to pulm circuit –> _____ –> Eisenmenger syndrome –> cyanosis

A

pulm HTN

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4
Q

Why do pts with Eisenmenger Syndrome get RVH?

A

increased pressure in pulmonary vascular circuit

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5
Q

Why do pts with Eisenmenger Syndrome get polycythemia?

A

deoxygenated blood in the systemic circuit causes hypoxemia –> epo released

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6
Q

Why do pts with Eisenmenger Syndrome get clubbing?

A

systemic cyanosis

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7
Q

What is the most common type of ASD?

A

ostium secundum

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8
Q

Which ASD is associated with Down Syndrome?

A

ostium primum

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9
Q

What direction is the blood shunted in ASD?

A

L to R

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10
Q

What heart sounds are heard with ASD?

A

split S2

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11
Q

What is a complication of ASD?

A

paradoxical emboli

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12
Q

PDA is associated with ____.

A

congenital rubella

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13
Q

____ is associated with congenital rubella.

A

PDA

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14
Q

The ductus arteriosus connects the ____ to the _____.

A

pulmonary artery to the aorta

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15
Q

The _____ connects the pulmonary artery to the aorta in fetal life.

A

ductus arteriosus

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16
Q

Which way does blood flow in the fetal ductus arteriosus?

A

from L to R (aorta to pulm artery)

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17
Q

When will a PDA present? How?

A

later in life with lower extremity edema

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18
Q

What is the murmur for PDA?

A

machine-like holosystolic murmur

19
Q

Why is the cyanosis in PDA in the lower extremities and not the upper?

A

bc the ductus arteriosus connects to the aorta after the aortic arch and its branches

20
Q

What is the tx for PDA? How does it work?

A

indomethacin

decreases PGE

21
Q

What keeeeeps the PDA open?

A

prostaglandin E (PGE)

22
Q

Which direction is the blood shunted in Tetrology of Fallot?

A

R to L

23
Q

How and when does Tetrology of Fallot present?

A

cyanosis at birth (early)

24
Q

Dx?

Juv pt squats after running around

A

Tetrology of Fallot

25
Q

Why does squatting help Tetrology of Fallot pts feel better?

A

it increases arterial vascular resistance, preventing blood from entering the aorta (decreased shunting) and pushes it instead into the lungs (increased afterload)

26
Q

Dx?

boot-shaped heart on CXR

A

Tetrology of Fallot

27
Q

What is the tx for transposition of the great vessels?

A

create a shunt

ex: keep PDA open with PGE

28
Q

What causes transposition of the great vessels?

A

maternal diabetes

29
Q

How does transposition of the great vessels present?

A

early cyanosis

30
Q

What problem in embryology causes truncus arteriosus?

A

the truncus fails to divide

31
Q

How do pts with truncus arteriosus present?

A

early cyanosis

32
Q

What is the problem in truncus arteriosus?

A

there is not separation of the pulm artery and aorta- there’s just one huge vessel coming off the ventricles

33
Q

What causes tricuspid atresia?

A

failure of the tricuspid valve orifice to develop (and therefore the R ventricle, too)

34
Q

What other problem is associated with tricuspid atresia?

A

ASD

35
Q

How do tricuspid atresia pts present?

A

early cyanosis

36
Q

What is coarctation of the aorta?

A

narrowing of the aorta

37
Q

The infantile form of coarctation of the aorta is associated with _____.

A

a PDA (Turner’s Syndrome)

38
Q

Where is an infantile coarctation of the aorta found?

A

distal to the aortic arch but proximal to the PDA

39
Q

How does infantile coarctaton of the aorta present?

A

as lower extremity cyanosis

40
Q

What heart findings are present in Turner syndrome?

A

infantile coarctation of the aorta
bicuspid aortic valve
PDA

41
Q

How does adult form coarctation of the aorta present?

A

HTN in upper extremities and hypotension in lower extremities

42
Q

What is the adult form of coarctation of the aorta associated with?

A

bicuspid aortic valve

43
Q

What is the CXR finding for the adult form of coarctation of the aorta?

A

notched ribs