Surgical management of pulmonary atresia with intact ventricular septum. Right ventricular size as a guideline for surgical intervention.

61 infants with critical valvular pulmonary stenosis (21 cases) or pulmonary atresia with intact ventricular septum (40 cases) were operated on between 1975-1989 in the Semmelweis University Medical School. Right ventricular volume, area, outflow tract dimension and tricuspid anulus diameter was measured by angiocardiography and echocardiography to evaluate right ventricular size, and to predict the operative outcome using these data. Our results suggested, that if the normalized right ventricular volume was less than 3 ml/m2, the normalized right ventricular area was less than 2.5 cm2/m2, and the normalized right ventricular area was less than 2.5 cm2/m2 and the normalized tricuspid anulus diameter was less than 1.2 cm2/m2/3 only a systemopulmonary shunt procedure is needed. In all other cases pulmonary valvotomy is necessary to decompress the right ventric and to help increase the right ventricular size. Where a different part of the right ventricle is hypoplastic a systemopulmonary shunt procedure is needed too. In the follow up period the right ventricular dimension and tricuspid valve diameter was measured by echocardiography. This noninvasive assessment can predict the preoperative diagnosis, postoperative outcome and demonstrates an adequate growth of the right ventricle after pulmonary valvotomy.