Tricuspid atresia

Background

Tricuspid atresia.svg
  • A cyanotic congenital heart defect
  • Absence of tricuspid valve resulting in abscess of communication between the RA and RV
  • Third most common cyanotic congenital heart disease [1]

Associated cardiac lesions

Physiology

  • Blood from the RA must exit through the ASD (there’s no tricuspid valve)
  • Right to left atrial shunting allows deoxygenated systemic venous blood to enter the LA, then LV
  • Cyanosis is present given mixing of systemic and pulmonary venous blood in the LA

Clinical Features

  • 50% present on the first day of life, additional 30% present by 1 month of age
  • Central cyanosis is the most notable feature on physical exam
  • Holosystolic murmur at left lower sternal border if a VSD is present
  • Continuous murmur if there is a PDA
  • Jugular venous distention and prominent “a” wave and hepatomegaly if there is a restrictive atrial level communication
  • Tachypnea may be present in patients with unrestrictive pulmonary blood flow

Differential Diagnosis

Congenital Heart Disease Types

Evaluation

  • Echocardiography
  • Chest x-ray
    • May have a paucity of pulmonary markings and normal heart size in patients with normal pulmonary blood flow (no VSD)
    • May have cardiomegaly and prominence of pulmonary vascularity in patients with increased pulmonary blood flow (large VSD)
  • ECG
    • May demonstrate tall P waves, left axis deviation, LVH, and diminished RV forces

Management

  • Stabilize cardiopulmonary function prior to surgery
  • Supplemental oxygen
  • Mechanical ventilation
  • Inotropic agents (eg, dopamine and dobutamine) improve myocardial contractility
  • Prostaglandin E1
    • Maintain adequate ductal dependent pulmonary flow
    • Start infusion at 0.05 mcg/kg/min IV and titrate up to 0.1 mcg/kg/min, monitoring for hypotension and apnea
    • Side Effects: Hypotension, Bradycardia, Seizures and Apnea
  • Staged surgical repair

Disposition

  • Admit

See Also

External Links

References

  1. Reller MD. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153(6):807-13.
  2. Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease