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.Pathology 2 > Congenital Heart Disease > Flashcards

Congenital Heart Disease Flashcards

1
Q

____ is the greatest risk factor for having a congenital heart disease

A

defect in sibling or parent is the greatest risk factor for having a congenital heart disease

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2
Q

____ is associated with coarctation of the aorta

A

Turner syndrome is associated with coarctation of the aorta

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3
Q

describe the fetal to adult circulation transition

A
  • at birth, the baby breathes, the lungs inflate and pulmonary resistance (right sided pressure) falls
  • blood flow stops through foramen ovale (flap valve)
  • ductus arteriosus closes by 15 hours of life
  • prostaglandins (PGs) maintain patency (keep open)
    • PGs produced in placenta, metabolized in lungs
    • at birth, reduced production/increased breakdown
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4
Q

there is an increase in _____ with left to right shunting of blood (plethoric lung fields) and a decrease in ____ with a right to left shunting of blood (oligemic lung fields)

A

there is an increase in lung vascularity with left to right shunting of blood (plethoric lung fields) and a decrease in vascularity with a right to left shunting of blood (oligemic lung fields)

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5
Q

____ is the most common form of cyanotic congenital heart disease

A

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease

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6
Q

name the 4 features of ToF

A

PROVe

  • pulmonary stenosis (infundibular stenosis): the degree determines the prognosis
  • right ventricular hypertrophy
  • overriding aorta
  • VSD
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7
Q

___ is associated with Tetralogy of Fallot

A

Down’s syndrome is associated with Tetralogy of Fallot

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8
Q

list the clinical features of ToF

A
  • usually present by 6 months
  • dyspnea on exertion, cyanosis, squatting
  • polycythemia → cerebral thrombosis
    • bc of persistent hypoxia → increased EPO → increased RBCs
  • IE
  • pulmonary vasculature decreased
  • associated with Down’s Syndrome
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9
Q

describe transposition of the great arteries

A
  • aorta off RV and pulmonary artery off LV
  • incompatible with post-natal life, unless a shunt (VSD or PDA) is present
  • the dominant clinical finding is cyanosis
  • the outcome depends on the degree of blood “mixing,” tissue hypoxia and the ability of the RV to maintain the systemic circulation
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10
Q

describe what is seen in the image

A

transposition of the great vessels

RV is much thicker than LV because the RV needs to pump the blood to the systemic circulation, whereas LV pumps to pulmonary circulation (lower pressure)

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11
Q

describe truncus arteriosus

A
  • failure of partitioning of embryologic truncus into aorta and pulmonary artery
  • single great artery gets blood from both ventricles
  • underlying VSD
  • blood from both ventricles mixes
  • cyanotic
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12
Q

describe tricuspid atresia

A
  • complete occlusion of the tricuspid orifice
  • underdeveloped RV (hypoplastic)
  • mitral valve larger than normal
  • R to L shunt through ASD (bypass obstruction)
  • L to R shunt through VSD (gets blood in RV)
  • cyanotic from birth with high mortality
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13
Q

describe total anomalous pulmonary venous connection (TAPVC)

A
  • pulmonary veins fail to join the left atrium
  • pulmonary return vessels drain into the left innominate vein or coronary sinus
  • ASD always present, allowing oxygenated pulmonary blood to return into the LA
  • R to L shunting through ASD due to lower LA pressure (no normal return) results in cyanosis
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14
Q

____ is the most common congenital heart lesion and is associated with ____

A

VSD is the most common congenital heart lesion and is associated with trisomy 21, 13, 18

the membranous portion is most commonly affected

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15
Q

name clinical features and complications of VSD

A
  • clinical features:
    • pulmonary HTN (bc RV receiving blood from RA + LV)
    • CHF
    • pansystolic murmur
  • complication:
    • shunt reversal leads to cyanosis = Eisenmenger complex
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16
Q

describe ASD

A
  • age at presentation variable, may be asymptomatic into adulthood
  • most common congenital malformation diagnosed in adults
  • pulmonary vascularity increased if significant left to right shunt
17
Q

describe the 3 types of ASD

A
18
Q

describe what is seen in the image

A
19
Q

describe patent ductus arteriosus (PDA)

A
  • females > males
  • continuous, “machine-like” murmur
  • may need to keep open with prostaglandin E (kEEp it open) if associated with other malformations
    • close with indomethacin (NSAID = prostaglandin inhibitor)
  • shunt may reverse leading to cyanosis
20
Q

describe the pathology of PDA

A
  • connect left pulmonary artery to aortic arch
  • closes with high O2 tension
  • higher incidence in maternal rubella infxn
  • associated with polycythemia
21
Q

describe coarctation of the aorta and name the 2 types

A
  • narrowed aortic lumen
  • associated with Turner’s syndrome
  • 2 types:
    • preductal (infantile): has PDA
    • postductal (adult): ductus arteriosus closed
22
Q

describe what is seen in the image

A

coarctation of the aorta

23
Q

describe clinical features of preductal coarctation of the aorta

A
  • preductal (presents in infancy)
    • CHF
    • selective cyanosis of lower extremities (PDA)
    • femoral pulses are weaker than those of the upper extremities
24
Q

describe clinical features of postductal coarctation of the aorta

A
  • postductal: presents in older children and adults
    • no selective cyanosis is seen
    • HTN of the upper extremities
    • BP is low and pulses are weak in lower extremities
    • notching of ribs due to collaterals
    • intermittent claudication; arterial insufficiency
25
Q

describe pulmonary valve stenosis and atresisa\

A
  • obstruction at pulmonary valve
  • stenosis results in RVH
  • atresia (no communication between RV and lungs)
    • hypoplastic RV and ASD
    • blood reaches lungs through PDA (L to R)
26
Q

describe aortic stenosis and the 3 types

A
  • 3 types of stenosis based on location:
    • valvular: affects valve cusps
    • subvalvular: “subaortic stenosis” a ring of fibrous tissue below valve cusps (results in LVH)
    • supravalvular: inherited aortic dysplasia (elastin defect) involves ascending aorta-thickened wall → luminal obstruction
27
Q

describe aortic atresia (hypoplastic left heart syndrome)

A
  • complete obstruction of aortic outflow leads to hypoplasia of the LV and aorta
  • dense endocardial fibroelastosis of underdeveloped LV
  • must have PDA to provide coronary blood flow
  • very severe cardiac malformation with early death

.Pathology 2 (28 decks)

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