Our surgical experience involving pulmonary atresia with intact ventricular septum (PPA) between the years 1981 and 1989 is reviewed. Twelve infants aged 3 days to 11 months were studied for right ventricular growth potential with angiocardiography and hemodynamics after transventricular pulmonary valvotomy (TVPV). Ten infants were divided into two groups, 5 infants (Group I) were treated with TVPV only, and the other 5 infants (Group II) required additional surgery after the initial operation (TVPV in 2 cases and TVPV with shunt in 3 cases). Post-operative RV growth potential was evaluated with right ventricular index (RVI) based on the tricuspid valve annulus, right ventricular inlet, and right ventricular outlet dimensions, and with tricuspid annular index (TVI) based on the tricuspid valve annulus normalized by aortic diameter. Follow-up (3-69 months after initial operation) studies demonstrated that the RV cavity increased in Group I (RVI of 15.75 +/- 2.92 preoperatively versus 17.00 +/- 1.57 postoperatively, TVI of 3.04 +/- 0.63 versus 3.85 +/- 0.40). In contrast, the RV cavity in Group II demonstrated a lack of growth (RVI of 9.24 +/- 2.60 versus 9.85 +/- 2.76, TVI of 1.46 +/- 0.46 versus 1.70 +/- 0.80). PPA infants with RVI greater than 11 have been treated successfully with TVPV alone, which may maximize the potential for RV growth, but infants with RVI less than 11 have not experienced RV growth + postoperatively and, therefore, have required second intracardiac operations.
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