Outcome 50 years after surgical repair of pulmonary valve stenosis: a longitudinal cohort study

Objectives Data on long-term outcomes after surgical repair of pulmonary valve stenosis are limited. This study evaluated survival, clinical outcomes and quality of life (QoL) after surgery during childhood.

Methods Single centre, longitudinal cohort study evaluating consecutive patients with pulmonary valve stenosis who underwent surgical repair between 1968 - 1980 and were evaluated every decade since 1990. Results Of the original cohort of 89 operated patients, 11 died (12%), including 2 who died within 30 days postsurgery (2%), and 7 (8%) were lost to follow-up.

Survival at 50 years follow-up was 87%, which was not significantly different from the GDP. Of the remaining 71 survivors, 32 refrained earlier from participating in this cohort study, leaving 39 eligible, of whom 34 (87%) participated again (50% male, median age 48 years) with a median follow-up of 45 (range 40 - 52) years.

Event-free survival was 50%, with supraventricular tachycardia (14%) and reintervention (13%) being the most frequent events, although less frequently in the last 10 years. At last follow-up, biventricular function was preserved in most patients.