OUTCOME MEASURES FOR THE NEONATAL MANAGEMENT OF PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

The outcome in 135 patients with pulmonary atresia with intact ventric ular septum was reviewed in terms of actuarial survival and ''suitabil ity'' for eventual definitive repair (defined as having a tricuspid va lve diameter larger than 2.4 standard deviations below the mean normal beyond the neonatal period). Of patients who underwent an initial clo sed valvotomy, 50% were dead, 22% were alive and suitable, and the rem aining 28% were alive but unsuitable for definitive repair at 5 years. None of 66 patients who underwent primary shunt alone achieved suitab ility and 52% were dead at 5 years. No mode of neonatal closed valvoto my was consistently satisfactory: in only 10 of 26 survivors with seri al measurements did the neonatal valvotomy alone achieve decompression to subsystemic pressures. Only half of the decompressed ventricles ac hieved growth of the tricuspid valve disproportionate to somatic growt h as a result of the neonatal procedure. Strategies for the neonatal t reatment of pulmonary atresia with intact ventricular septum should no t rely on closed operations on the pulmonary valve to optimize right v entricular growth.