Tricuspid Atresia.

Untreated, the prognosis for patients with tricuspid atresia (TA) is poor. Recent advances in medical and surgical therapy, particularly the application of Fontan principle, have markedly improved the long-term outlook for children with this condition. Palliative procedures to normalize the pulmonary blood flow and to relieve interatrial or interventricular obstruction should be undertaken promptly. Staged total cavopulmonary connection to bypass the right atrium and right ventricle by an initial bidirectional Glenn procedure and followed by extracardiac conduit diversion of inferior vena caval flow into the pulmonary arteries appears to be the current procedure of choice in the surgical management of TA. Total cavopulmonary diversion appears to be superior to conventional Fontan-Kreutzer operations, but long-term follow-up results are needed to confirm this impression.