Cj. Shanley et al., PRIMARY UNIFOCALIZATION FOR THE ABSENCE OF INTRAPERICARDIAL PULMONARY-ARTERIES IN THE NEONATE, Journal of thoracic and cardiovascular surgery, 106(2), 1993, pp. 237-247
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.