PRIMARY UNIFOCALIZATION FOR THE ABSENCE OF INTRAPERICARDIAL PULMONARY-ARTERIES IN THE NEONATE

The management of the neonate with absence of intrapericardial pulmona ry arteries in association with complex intracardiac anomalies present s a challenging surgical problem. The more traditional approach of pal liation with unilateral or bilateral systemic-pulmonary artery shunts may result in peripheral pulmonary artery stenoses and uneven distribu tion of pulmonary blood flow. In addition, this approach may lead to c omplicated reconstructive procedures necessitating reconstruction of t he branch pulmonary artery with prosthetic material, which restricts p ulmonary artery growth and often complicates reoperation. To avoid the se potential limitations, we have performed primary unifocalization fo r absence of intrapericardial pulmonary arteries in eight consecutive neonates (median age 9 days) between May 1990 and December 1991. Absen ce of intrapericardial pulmonary arteries occurred in association with tetralogy of Fallot (n = 4), truncus arteriosus (n = 2), and transpos ition of the great arteries with pulmonary atresia (n = 2). Four patie nts had unilateral absence of the right (n = 1) or left (n = 3) intrap ericardial pulmonary artery. In the remaining four patients, there was complete absence of both intrapericardial pulmonary arteries. Wide mo bilization and excision of all ductal tissue before anastomosis was pe rformed from a midline approach in seven patients. In one patient, a p reliminary right thoracotomy was required. Primary unifocalization was performed simultaneously with complete repair in five patients. In th e remaining three patients, unifocalization was part of a staged repai r and included insertion of a systemic-pulmonary artery shunt to the r econstructed central pulmonary artery confluence. No operative or late cardiac deaths occurred, although one death occurred during subsequen t repair of a tracheoesophageal fistula. Three patients underwent reop eration, and only one patient required revision of an anastomotic pulm onary artery stenosis. All survivors were growing normally at 2 to 22 months after operation (mean follow-up 10 months). Our experience sugg ests that primary reconstruction for the absence of intrapericardial p ulmonary arteries can be successfully accomplished in the neonate. Thi s approach provides uniform bilateral pulmonary blood flow, avoids pro sthetic material in the branch pulmonary arteries, and may eliminate, or at least simplify, future reconstructive procedures.