Primary unifocalization for the absence of intrapericardial pulmonary arteries in the neonate

The management of the neonate with absence of intrapericardial pulmonary arteries in association with complex intracardiac anomalies presents a challenging surgical problem. The more traditional approach of palliation with unilateral or bilateral systemic-pulmonary artery shunts may result in peripheral pulmonary artery stenoses and uneven distribution of pulmonary blood flow. In addition, this approach may lead to complicated reconstructive procedures necessitating reconstruction of the branch pulmonary artery with prosthetic material, which restricts pulmonary artery growth and often complicates reoperation. To avoid these potential limitations, we have performed primary unifocalization for absence of intrapericardial pulmonary arteries in eight consecutive neonates (median age 9 days) between May 1990 and December 1991. Absence of intrapericardial pulmonary arteries occurred in association with tetralogy of Fallot (n = 4), truncus arteriosus (n = 2), and transposition of the great arteries with pulmonary atresia (n = 2). Four patients had unilateral absence of the right (n = 1) or left (n = 3) intrapericardial pulmonary artery. In the remaining four patients, there was complete absence of both intrapericardial pulmonary arteries. Wide mobilization and excision of all ductal tissue before anastomosis was performed from a midline approach in seven patients. In one patient, a preliminary right thoracotomy was required. Primary unifocalization was performed simultaneously with complete repair in five patients. In the remaining three patients, unifocalization was part of a staged repair and included insertion of a systemic-pulmonary artery shunt to the reconstructed central pulmonary artery confluence. No operative or late cardiac deaths occurred, although one death occurred during subsequent repair of a tracheoesophageal fistula. Three patients underwent reoperation, and only one patient required revision of an anastomotic pulmonary artery stenosis. All survivors were growing normally at 2 to 22 months after operation (mean follow-up 10 months). Our experience suggests that primary reconstruction for the absence of intrapericardial pulmonary arteries can be successfully accomplished in the neonate. This approach provides uniform bilateral pulmonary blood flow, avoids prosthetic material in the branch pulmonary arteries, and may eliminate, or at least simplify, future reconstructive procedures.