6. Congenital Heart Diseases Flashcards

1
Q

Definition of congenital heart diseases

A

Abnormalities of the heart or great vessels that are present from birth

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2
Q

Types of congenital heart diseases

A
  1. Left-to-right shunt
    a. Atrial septal defect
    b. Ventricular septal defect
    c. Patent ductus arteriosus
  2. Right-to-left shunt
    a. Tetralogy of Fallot
    b. Transposition of the great vessels
    c. Tricuspid atresia
    d. Persistent truncus arteriosus
  3. Obstructive congenital anomalies
    a. Coarctation of aorta
    b. Pulmonary stenosis & atresia
    c. Aortic stenosis & atresia
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3
Q

Definition of Left-to-Right Shunts

A

Typically acyanotic at onset, but may become cyanotic with eventual pulmonary hypertension or shunt reversal

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4
Q

Atrial septal defect

A
  1. Abnormal, fixed opening in the atrial septum due to
    incomplete tissue formation
  2. Despite right sided volume overload, ASD is usually well totally and asymptomatic until adulthood
  3. Ejection systolic murmur (due to excessive flow through the pulmonary valve) + permanent splitting of S2
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5
Q

Ventricular septal defect

A
  1. Incomplete closure of the ventricular septum (mostly in the membranous portion, minority in the muscular part)
  2. Functional consequences depend on size of VSD
    - If small, asymptomatic until adulthood
    - If large, cause right ventricular hypertrophy &
    pulmonary hypertension virtually from birth, which may progress to irreversible pulmonary vascular disease and shunt reversal (Eisenmenger syndrome)
  3. Pan-systolic murmur
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6
Q

Patent ductus arteriosus

A
  1. Ductus arteriosus remaining open after birth
  2. Depending on diameter of PDA, may result in progressive
    obstructive changes in pulmonary vasculature (due to
    pulmonary volume overload)
  3. Continuous/harsh/machinery-like murmur
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7
Q

Definition of right-to-left shunts

A

Typically cyanotic

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8
Q

Tetralogy of fallot

A
  1. Four cardinal features that arises from an anterocephalad deviation of the anterior septum:
    - Ventricular septal defect
    - Aorta that overrides the VSD
    - Pulmonary stenosis
    - Right ventricular hypertrophy

PROVe - Pulmonary stenosis, Right ventricular hypertrophy, Overriding aorta, VSD

  1. Heart is enlarged & ‘boot-shaped’
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9
Q

Transposition of the great vessels

A
  1. Ventriculoarterial discordance: aorta originates from RV, pulmonary trunk from LV
  2. Due to abnormal formation of truncal septa
  3. Right ventricular hypertrophy & left ventricular atrophy
  4. Without surgical repair, patient dies within first few
    months of life
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10
Q

Tricuspid atresia

A
  1. Complete occlusion of the tricuspid valve orifice due to unequal division of the atrioventricular canal
  2. Concomitant ASD & VSD allows maintenance of
    pulmonary & systemic circulation
  3. High mortality within first few weeks or months of life
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11
Q

Persistent Truncus Arteriosus

A

Single great artery receiving blood from both ventricles

due to a failure of separation of truncus arteriosus into aorta & pulmonary trunk

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12
Q

Coarctation of the aorta

A
  1. A narrowing/constriction of the aorta which usually
    occurs distal to the branches of the arch of aorta
  2. Results in hypertension of the upper extremities and hypotension in the lower extremities (weak pulses, intermittent claudication, coldness)
  3. Left ventricular hypertrophy (due to pressure overload)
  4. Radiographically, notching of the undersurfaces of the ribs evident (due to dilation of intercostals arteries involved in anastomosis to bypass coarcation)
  5. Ejection systolic murmur
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13
Q

Pulmonary Stenosis & Atresia

A
  1. May occur in isolation or as part of a more complex
    anomaly (e.g. Tetralogy of Fallot)
  2. Right ventricular hypertrophy (due to pressure overload)
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14
Q

Aortic Stenosis & Atresia results in?

A

Left ventricular hypertrophy (due to pressure overload)

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