Updated: 5/2/2018

Other Cyanotic Congenital Heart Defects

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Snapshot
  • A newborn boy is born to a 25-year-old mother and noted immediately to be cyanotic. She had no prenatal care during her pregnancy. She has a past medical history of bipolar disorder and takes lithium inconsistently. However, she believes that she did take it during the pregnancy. On physical exam, the newborn has a holosystolic murmur. An echocardiogram reveals atrialization of the right ventricle and downward displacement of the tricuspid valve, as well as tricuspid regurgitation. (Ebstein anomaly)
Introduction
  • Cyanotic heart diseases
    • 5 T’s
      • Truncus arteriosus (1 vessel)
      • Transposition of the great vessels (2 vessels are switched)
      • Tricuspid atresia (3 = tri)
      • Tetralogy of fallot (4 = tetra)
      • Total anomalous pulmonary venous return (TAPVR = 5 letters)
    • Ebstein anomaly
    • may require atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosus (PDA) to survive
  • Clinical presentation
    • early cyanosis, resulting in “blue babies” soon after birth
      • depending on severity and type of the defect, cyanosis may not be apparently immediately after birth
    • symptoms of congenital heart disease
      • tachypnea
      • feeding difficulties
      • failure to thrive
      • cyanosis
    • heart failure
  • Imaging
    • chest radiography
      • findings vary based on lesion (see chart below)
    • echocardiogram
      • gold standard for diagnosis
 
Other Cyanotic Congenital Heart Defects
Heart Disease
Pathogenesis
Clinical Presentation
Diagnosis Notes
Truncus arteriosus
  • Single large vessel arising from the heart and supplying the body with mixed blood (oxygenated and deoxygenated blood)
    • secondary to failure of the aorticopulmonary septum to form
  • Systolic ejection murmur with thrill
  • Single S2 heart sound
  • High-pithced diastolic murmur
  • Initially only minimal cyanosis
  • Chest radiograph
    • cardiomegaly
    • ↑ pulmonary vasculature
  • Associated with DiGeorge syndrome
  • Treatment
    • corrective surgery
Tricuspid atresia
  • No tricuspid valve
  • Hypoplastic right ventricle
  • Requires patent foramen ovale, ASD, or VSD
  • Severe cyanosis at birth
  • Increased left ventricular impulse
  • Holosystolic murmur
  • Chest radiograph
    • ↓ pulmonary vasculature
  • Treatment
    • prostaglandin E1
    • corrective surgery
Total anomalous pulmonary venous return (TAPVR)
  • Pulmonary veins drain into right heart circulation
  • ASD or PDA allows for right-to-left shunting to maintain cardiac output
  • Pulmonary hypertension
  • Cyanosis depends on severity of obstruction
  • Chest radiograph
    • cardiomegaly
    • cardiac shadow has "snowman" appearance
    • ↑ pulmonary vasculature
  • Treatment
    • prostaglandin E1
    • emergent corrective surgery in severe cases
Ebstein anomaly
  • Characterized by a downardly displaced tricuspid valve, resulting in an enlarged right atrium
  • Increased right atrial pressure shunts blood to the left heart via te foramen ovale or ASD, leading to cyanosis
  • Tricuspid regurgitation
  • May present with cyanosis in newborns or present later in life
  • Holosystolic murmur
  • Right heart failure
  • Chest radiograph
    • cardiomegaly
    • ↓ pulmonary vasculature
  • Associated with lithium exposure in utero
  • Treatment
    • prostaglandin E1
    • corrective surgery
 
 

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