Fifty-Five Years Follow-Up of 111 Adult Survivors After Biventricular Repair of PAIVS and PS

There is paucity of long-term data on adult survivors after biventricular repair of pulmonary atresia with intact ventricular septum (PAIVS) and pulmonary stenosis (PS). This study aimed to determine the cardiac and non-cardiac outcomes of adult survivors after biventricular repair of PAIVS and PS. The cardiac, neurodevelopmental and liver problems of 111 adults, 40 with PAIVS and 71 with PS, were reviewed. The median follow-up duration of our patients was 26.5years (range 14.8-55years). The freedom from reintervention at 30years was 17.4% and 73.3% for PAIVS and PS patients (p<0.001), respectively. Compared with PS patients, PAIVS patients had significantly greater prevalence of right atrial and right ventricular (RV) dilatation, and moderate to severe tricuspid and pulmonary regurgitation (all p<0.05), and cardiac arrhythmias (22.5% vs. 8.5%, p=0.047). The freedom from development of cardiac arrhythmias at 30years of 68.4% and 91.6%, respectively, in PAIVS and PS patients (p=0.03). Cox proportional hazards model identified PAIVS as an independent risk factor for reintervention (HR 4.0, 95% CI 2.1-7.6, p<0.001) and development of arrhythmias (HR 4.1, 95% CI 1.1-14.4, p=0.03). Neurodevelopmental problems were found in 17.5% of PAIVS patients and 7.0% of PS patients (p=0.11). Liver problems occurred in 2 (5%) PAIVS patients, both of whom required conversion to 1.5 ventricular repair. In conclusion, long-term problems, including the need for reinterventions, cardiac arrhythmias, RV dilation, pulmonary regurgitation, and neurodevelopmental and liver issues are more prevalent in adult PAIVS than PS survivors.