Introduction
During the last 30 years the surgical strategy for pulmonary atresia
(PA) with ventricular septal defect (VSD) and systemic-pulmonary
collateral arteries (SPCA’s) is subject of ongoing discussion. There are
several reports using the multi-staged approach as well as reports on
two-staged approach or one-stage midline primary repair. [1-6]
Furthermore it has been reported that unifocalization possibly does not
bring long-term benefit in terms of late survival.[7]
Historically patients with PA, VSD and SPCA’s were treated in our
hospital with various surgical interventions depending on the clinical
condition and previous cardio-surgical procedures on presentation in our
clinic. Since 1989 a protocol as described previously was followed in
all our patients presenting with PA, VSD and SPCA’s.[8] This
protocol consisted of staged unifocalization procedures with a
subsequent total correction, with closure of the VSD and placing a
pulmonary homograft between the right ventricular outflow tract (RVOT)
and the pulmonary bifurcation. We report our results of 30 year
experience with this staged protocol applied to all consecutive
patients.