G. Favia Guarnieri, J.D. Piot, V. Lambert, A. Touchot, E. Belli, A. Serraf, C. Planché, J. Losay


Centre Chirurgical Marie-Lannelongue, Paris, France


     Pulmonary atresia with intact ventricular septum lacks an uniform surgical approach. The nature and the success of early management strategies are critical to subsequent changes in function of the right ventricle (RV) and suitability for definitive repair. The aim of this study was to analyse the midterm outcome of pts with PA-IVS who underwent pulmonary valvotomy (Pvy) in relation to neonatal echocardiographic data.

From 1990 to 2003, 45 pts were retrospectively studied. The median age at PVy was 6 days, median weight 3,1 kg. The exclusion criteria included a monopartite RV, RV-dependent coronary circulation and severe Ebstein anomaly. The mean tricuspid valve (TV) diameter was 10,2 mm (5-20) and  mean Z value was –2,26 (+2 to –6,5), mean TV/mitral valve (MV) ratio was 0,83 (.35-1.81). RV morphology was bipartite in 7 pts, tripartite in 38. Open PVy was performed in 33 pts as the initial procedure and Blalock-Taussig (BT) shunt was required in 30 of them 0 to 14 days after. Percutaneous PVy was performed in 12 pts and 6 of them required BT shunt 3 to 21 days after. Preinterventional echocardiograms were not different; BT was needed significantly more often after surgical PVy. There were 5 early  and 1 late sudden death. Among the survivors ( mean follow up 6 + 4.4 years) 27 pts had  a biventricular repair, 4 had one ventricular repair and 8 pts are awaiting for further surgery. Comparison of initial parameters with the most recent clinical status showed significant difference (p<.05) of the TV diameter (11.3 vs 7.5), RV lenght (19.6 vs 13), and TV/MV ratio (.9 vs .46). Percutaneous valvotomy may avoid the need of secondary BT.Neonatal echo parameters allow prediction of the pts who will have complete repair.