8.3 Congenital Defects Flashcards

1
Q

What heart defect to suspect:

-fetal alcohol syndrome

A

VSD

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1
Q

What heart defect to suspect:

-Bicuspid aortic valve

A

Coarctation of the Aorta, adult form

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1
Q

Transposition of the great vessels

  • what is it
  • Tx
A
  • aorta and pulm artery switch places. Lethal immediately after birth
  • Treat by surgery–switching places of vessels.
  • To keep infant alive before definitive surgery, do 2 things:
    1. PGE to keep PDA for blood flow
    2. surgical creation of septal shunts in heart
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1
Q

Truncus arteriosus

A
  • trunk of aorta/pulmonary artery fails to fully divide
  • causes cyanosis
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2
Q

What congential disorder is Transposition of the Great Vessels associated with?

A

-maternal diabetes

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3
Q

what congential disorder is ASD associated with?

A

Down’s (with less common ostium primum type of ASD)

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4
Q

What heart defect to suspect:

-Down syndrome

A

ASD (ostium primum type, not the more common ostium secondum)

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4
Q

Tx of PDA

A
  • Indomethacin, to decrease PGE
  • PDA is maintained by PGE (‘pgE kEEps PDA open’)
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5
Q

Coarctation of the Aorta

-describe both types

A

Aorta is narrowed.

  1. Infantile
    - coarctation occurs between PDA and aortic arch. Because of low pressure distal to coarctation, there is R-L shunt through the PDA, resulting in lower extremity cyanosis
  2. Adult
    - no PDA. HTN of upper extremities, hypotension of lower extremities (weak pulses). Blood will try to bypass coarctation through intercostal arteries, causing ‘notching of ribs’ by engorging those arteries.
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6
Q

Eisenmenger’s syndrome

-what results (4)

A
  1. cyanosis (reversal to R-L shunt)
  2. RV hypertrophy (from increase in pulm resistance)
  3. polycythemia (low pO2 increases EPO, increasing hematocrit)
  4. Clubbing (caused by cyanosis)
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8
Q

Clinical progression, symptoms of PDA

A
  1. asymptomatic at birth
  2. eventual Eisenmenger’s syndrome, results in cyanosis of lower extremities b/c PDA is located distal to aortic arch
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9
Q

What congential disorder is PDA associated with?

A

Congenital rubella (german measles)

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10
Q

what does PDA murmur sound like?

A

-constant, machine-like murmur

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11
Q

What heart feature is Coarctation of the Aorta (adult form) associated with?

A

bicuspid aortic valve

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12
Q

Tetralogy of Fallot

  • what do patients learn to do
  • characteristic X Ray finding
A
  1. pts learn to squat to increase arterial resistance, pushing back the shunted blood towards pulmonic circulation
  2. Boot shaped heart
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13
Q

What congential disorder is Coarctation of the Aorta (infant form) associated with?

A

Turner’s

14
Q

baby with lower extremity cyanosis:

-what to suspect?

A

Think R-L shunt through PDA (distal to aortic arch)

  1. Coarctation of the Aorta (infantile)
  2. PDA with Eisenmenger’s syndrome
16
Q

Suspect what? Adult on physical exam:

-you find strong pulses in upper extremities but weak pulses in lower extremities

A

Coarctation of the Aorta (adult)

17
Q

Tetralogy of Fallot

  • those 4 things
  • which one determines severity of disorder
A
  1. RV stenosis
  2. VSD
  3. Aorta attaches to VSD
  4. RV hypertrophy
    - the RV stenosis level determines severity by determining how much blood is shunted to the VSD aorta (causing cyanosis).
18
Q

What heart defect to suspect:

-congenital rubella (german measles)

A

PDA

19
Q

tricuspid atresia

A
  • failure of formation of tricuspid valve (blood can not enter RV)
  • often associated with a ASD (where else will the blood go) , so you get R-L shunt, early cyanosis
20
Q

What weeks in embryogenesis do cardiac defects usually occur?

A

weeks 3-8

21
Q

what is most common congenital heart defect?

A

VSD

23
Q

ASD

  • types
  • clinical finding on physical exam
  • complications
A
  1. Ostium secundum (90%)
  2. Ostium primum (associated with Down’s)
    - fixed splitting of S2, independent of inspiration. Pulmonic valve closes later, from extra blood.
    - can eventually cause Eisenmenger’s syndrome
    - also possible paradoxical emboli
25
Q

What heart defect to suspect:

-Turner syndrome

A

Coarctiation of the Aorta (infant form)

26
Q
  • What can VSD eventually cause?
  • what congential disorder is VSD associated with?
A
  • Large defects can lead to Eisenmenger’s syndrome
  • fetal alcohol syndrome
27
Q

What heart defect to suspect:

-maternal diabetes

A

Transposition of great vessels

28
Q

Left to right congenital shunt:

-general progression

A
  1. may be asymptomatic at birth
  2. Eisenmenger’s syndrome: Reversal of shunt can occur from increased pulmonary resistance. This causes cyanosis, R ventricular hypertrophy, polycythemia, and clubbing