Congenital CV Abnormalities - Severson Flashcards

1
Q

What is ectopia cordis, and what is believed to cause this condition?

A
  • congenital malformation in which the heart is abnormally located either partially or totally outside of the thorax
    • due to failure in folding of the lateral body wall and its meeting with the cephalic fold
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2
Q

How does the dextrocardia develop?

A
  • abnormal folding of the embryonic heart
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3
Q

What developmental structures and events are responsible for an Atrial Septal Defect?

A
  • Excessive resorption of 1° septum
  • Incompetent foramen ovale
    • due to hypoplastic growth of 2° septum
  • Inadequate development of the primary septum
    • produces basal opening
  • Sinus venosus development defect
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4
Q

How do Atrial Septal Defects (ASDs) manifest after birth?

A
  • Left → Right shunting
  • increased pulmonary flow
    • only large ones lead to pulmonary hypertension with cyanosis tardive and cardiac failure
  • Right ventricular failure
  • Paradoxical embolism
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5
Q

What developmental abnormalities occur in ventricular septal defects (VSD)? How are they clinically manifested?

A
  • 90% due to malformation of membranous portion of ventricular septum
  • Muscular septum involvement may exhibit multiple sites
    • swiss-cheese septum
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6
Q

How are Ventricular Septal Defects clinically manifested?

A
  • Size dependent
  • Pulmonary stenosis
  • Murmur
  • Cyanosis tardive in long-standing cases
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7
Q

What is the role of the bulbar/truncal ridges (aorticopulmonary septum) in the development of Persistent Truncus Arteriosus?

A
  • complete developmental failure of the bulbar & truncal ridges
    • aorticopulmonary septum never forms
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8
Q

What is the role of the bulbar/truncal ridges (aorticopulmonary septum) in the development of Transposition of the Great Arteries?

A
  • failure of the normal spiraling of the aorticopulmonary spetum
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9
Q

What is the role of the bulbar/truncal ridges (aorticopulmonary septum) in the development of Tetralogy of Fallot?

A
  • anterior malalignment of the aorticopulmonary septum
  • causes tetrad of abnormalities
    • VSD
    • pulmonary stenosis (tiny pulmonary trunk)
    • overriding (large) aorta
    • right ventricular hypertrophy develops progressively from resistance to blood flow through the right ventricular outflow tract.
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10
Q

What are the two types of Coarctation of the Aorta that can occur?

A
  • Postductal (adult form)
    • 95%
    • 2xMales vs. females
  • Preductal (infantile form)
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11
Q

What are the clinical manifestations of a Postductal Coarctation of the Aorta?

A
  • Increased BP in arms
  • Decreased BP in legs
  • Notched ribs
    • enlarged intercostal/interthoracic arteries
  • Weak pulses in lower limbs
  • Complications:
    • CHF
    • CVA
    • rupture
    • infection
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12
Q

What are the clinical manifestations of a Preductal Coarctation of the Aorta?

A
  • Patent ductus arteriosus
  • Early CHF due to hypertrophy of R. ventricle
  • Cyanosis in lower half of body
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13
Q

What clinical situations may prevent closure of the Ductus Arteriosus?

A
  • Transposition of the Great Arteries
  • Pulmonary stenosis & Atresia (orifice closed)
  • Coarctation of Aorta
  • Aortic stenosis, & Atersia
  • Preterm birth (lungs not developed)
  • Congenital Rubella Syndrome
  • Down Syndrome
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14
Q

How might a Patent Ductus Arteriosus be treated?

A
  • Premies/Newborns:
    • Indomethacin up to 1 week
  • 1+ week Postnatal:
    • Surgery with catheter-placed occluder
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15
Q

What are the clinical features of Patent Ductus Arteriosus?

A
  • “Machine-like” murmur
  • Maternal rubella (german measles) during early weeks of pregnancy
  • Infectious endocarditis
  • Pulmonary hypertension
  • Slightly enlarged liver
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16
Q

What cardiac abnormalities are associated with Right-to-Left shunts?

A
  • Tetralogy of Fallot
  • Transposition of the Great Arteries
  • Truncus Arteriosus

(“Blue babies” take take take oxygen right away!)

17
Q

What cardiac abnormalities are associated with Left-to-Right Shunts?

A
  • Atrial Septal Defect
  • Ventricular Septal Defect
  • Patent Ductus Arteriosus

(late cyanosis = “blue kids”)

18
Q

What developmental structure is responsible for the occurrence of either pulmonary or aortic stenosis?

A

Defective development of aorticopulmonary septum (bulbar-truncal ridges)