Late Right Ventricular Reconstruction Following Valvotomy in Pulmonary Atresia with Intact Ventricular Septum

Experience over two decades in the surgical management of pulmonary atresia with intact ventricular septum demonstrates that eventual right ventricular (RV) reconstruction is possible in the majority of patients surviving valvotomy in infancy. Ten of 17 operative survivors of early valvotomy have eventually received a patch graft to the RV outflow tract, with no reoperative deaths (mean follow-up, 7.4 years). RV systolic pressures, suprasystemic prior to reoperation, are near normal after outflow patch reconstruction. Serial cineangiograms show evidence of RV growth by measurement of tricuspid annulus diameter (TAD), and demonstrate a rate of growth [d(TAD)/d(body length)] greater than a normal rate derived from autopsy data. The mean TAD growth rate is significantly greater than that of patients with less favorable ventricle types treated with a systemic-pulmonary shunt alone. Measurement of TAD is a useful method for following RV growth, and may aid in selecting patients for RV reconstruction.