Management of a patient with tricuspid atresia within the first month of life may include:

A. Creation of a systemic artery–to–pulmonary artery shunt.

B. Observation.

D. Pulmonary artery banding.

DISCUSSION: Initial management of newborn infants with tricuspid atresia is determined by the anatomic and physiologic factors that affect the balance of pulmonary and systemic blood flow. Infants with severely limited pulmonary blood flow and arterial oxygen saturations of less than 70% should be stabilized with PGE 1 to maintain patency of the ductus arteriosus until a systemic-to-pulmonary artery shunt can be performed. Patients with unobstructed pulmonary blood flow may exhibit only mild cyanosis but suffer from significant congestive heart failure. Many of these patients are best managed by pulmonary artery banding to decrease the volume overload on the left ventricle and to prevent the early development of irreversible pulmonary vascular disease. Some patients with moderate restriction of pulmonary blood flow may have balanced delivery of blood to the systemic and the pulmonary circulation. These patients can be carefully followed until such time as an imbalance develops or they become candidates for a bidirectional superior cavopulmonary (Glenn) anastomosis or a Fontan procedure. The normally high pulmonary vascular resistance present in the first month of life precludes the performance of either the Glenn or the Fontan procedure in the newborn.