TETRALOGY OF FALLOT WITH ABSENT PULMONARY VALVE - A CONTINUING CHALLENGE

Absent pulmonary valve syndrome (APVS); the combination of tetralogy o f Fallot (TOF) with agenesis of the pulmonary valve, is a relatively r are cardiac malformation. Despite the anatomic similarity with classic TOF, the pathophysiology is strikingly different. Data on 10 patients (3 male, 7 female) with APVS, treated between January 1978 and Decemb er 1995, were retrospectively reviewed. During this period a total of 2920 children underwent correction of a variety of congenital cardiac anomalies, of which 246 patients (8%) had a correction for TOF. Two pa tients with APVS presented within the first four months of life with s evere cardiorespiratory distress and required several operative proced ures. The remaining eight patients had only mild to moderate respirato ry and/or cardiac symptoms and elective intracardiac repair was perfor med on those between the ages of 10 months and 9.5 years. Associated c ardiac anomalies seen in five patients included aberrant coronary arte ry, absent or interrupted left pulmonary artery, partial AVSD and aber rant azygos continuation. In those electively corrected, the strategie s used were ventriculotomy (7), pulmonary homograft (3) and aneurysmor rhaphy (2). There were two deaths, one in each group of patients, as a result of progressive respiratory insufficiency and cardiac tamponade , respectively. The follow-up of the eight survivors ranged from 2 to 11 years (median 6.75). All have a normal effort tolerance; only one c hild is on digoxin therapy, and one child continues to suffer bronchos pastis episodes. Our experience with infants with this lesion is limit ed but underlines the different approaches required, depending on the age of presentation.