Tricuspid atresia


Tricuspid atresia.svg
  • A cyanotic congenital heart defect
  • Absence of tricuspid valve resulting in absence of communication between the right atrium and right ventricle
  • Due to absence of communication with the right ventricle, RV becomes either under-developed or entirely absent
  • Third most common cyanotic congenital heart disease [1]

Associated cardiac lesions


  • Blood from the RA must exit through the ASD (no tricuspid valve)
  • Right to left atrial shunting allows deoxygenated systemic venous blood to enter the LA, then LV
  • Cyanosis is present due to 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 and can be progressive
  • May also present with poor feeding
  • Holosystolic murmur at left lower sternal border if 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
  • EKG may show left axis deviation and LVH
  • Heart size is normal typically

Differential Diagnosis

Congenital Heart Disease Types


  • 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


  • 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


  • Admit

See Also

External Links


  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.