PubMed1985
The courses of 22 consecutive patients with pulmonary atresia and intact ventricular septum who underwent surgery between 1977 and 1984 were reviewed. This included 18 patients undergoing an initial surgical palliation, and four patients referred for a definitive procedure. A surgically oriented classification based on the degree of right ventricular hypoplasia was developed and applied. Three groups were identified and were termed the mild, moderate, and severe hypoplasia groups. Those in the mild and moderate groups had mild or moderate right ventricular and tricuspid hypoplasia with well-developed right ventricular outflow tracts and were therefore acceptable candidates for procedures to create an opening between the right ventricle and pulmonary artery. Those in the severe hypoplasia group and severe hypoplasia of the tricuspid valve, the right ventricle, and the right ventricle outflow tract so that attempts at establishing continuity with the pulmonary artery were thought to be unlikely to succeed. There were three patients with mild, 11 with moderate, and eight with severe hypoplasia. In the mild hypoplasia group, all three patients initially underwent valvotomy alone, but two required a shunt in the early postoperative period. In the moderate hypoplasia group, all patients underwent a valvotomy and received a central shunt with a snare, which allowed subsequent adjustment of pulmonary flow without thoracotomy in four patients. In the severe hypoplasia group, five patients received a shunt alone and one underwent valvotomy with atrial septectomy. This last patient represents the only early (less than 30 days) death in the series (6% mortality).(ABSTRACT TRUNCATED AT 250 WORDS)