Rational Approach to the Surgical Management of Tricuspid Atresia

A rational approach to the surgical management of tricuspid atresia with diminished blood flow to the lungs is as follows: A cava-pulmonary artery shunt is performed as the initial operation whenever possible. In the newborn infant and in patients where the pulmonary artery is less than one-half the diameter of the superior vena cava where the two vessels cross, the right pulmonary artery is anastomosed end-to-side to the ascending aorta. Subsequently, when the right pulmonary artery has enlarged, it is detached from the aorta and anastomosed to the superior vena cava. Closure of the outflow from the right ventricle to the left lung can be expected in tricuspid atresia after the first year of life, and unless the ductus remains patent, a systemic artery-pulmonary artery shunt on the left side will be required to supplement the cava-pulmonary artery shunt. This shunt will also be required as a preliminary step to replacing the ascending aorta-right pulmonary artery shunt with a cava-right pulmonary artery shunt if blood flow to the left lung is by bronchial collateral arteries only. Banding of the pulmonary artery is indicated in those patients with excessive blood flow to the lungs. In the present series of 19 patients with tricuspid atresia, there were 17 with diminished flow to the lungs and two with excessive flow. Eight patients with small pulmonary arteries had a shunt made between the ascending aorta and right pulmonary artery. Five patients were less than two weeks of age. All survived operation; one died a year postoperatively of unknown causes. Two shunts have been replaced with cava-pulmonary artery shunts. Ten patients had a shunt made between the superior vena cava and the right pulmonary artery one to eight years ago. There has been no operative or late mortality. A supplemental systemic artery-pulmonary artery shunt on the left side has been established in three patients. Two patients have had operative enlargement of the interatrial communication. Excessive blood flow to the lungs was present in two patients. Neither patient survived banding of the pulmonary artery.