TY - JOUR T1 - The unnatural history of pulmonary stenosis up to 40 years after surgical repair JF - Heart JO - Heart SP - 273 LP - 279 DO - 10.1136/heartjnl-2015-309159 VL - 103 IS - 4 AU - Judith A AE Cuypers AU - Myrthe E Menting AU - Petra Opić AU - Elisabeth M WJ Utens AU - Willem A Helbing AU - Maarten Witsenburg AU - Annemien E van den Bosch AU - Ron T van Domburg AU - Sara J Baart AU - Eric Boersma AU - Folkert J Meijboom AU - Ad J JC Bogers AU - Jolien W Roos-Hesselink Y1 - 2017/02/15 UR - http://heart.bmj.com/content/103/4/273.abstract N2 - Objective To provide prospective information on long-term outcome after surgical correction of valvular pulmonary stenosis (PS).Methods Fifty-three consecutive patients operated for PS during childhood between 1968 and 1980 in one centre are followed longitudinally for 37±3.4 years, including extensive in-hospital examination every 10 years.Results Survival information was available in 100% of the original 53 patients. Cumulative survival was 94% at 20 years and 91% at 40 years. Excluding perioperative mortality (<30 days), survival was 94% at 40 years. Of 46 eligible survivors, 29 participated in the in-hospital examination and 15 gave permission to use their hospital records (96% participation). Cumulative event-free survival was 68% after 40 years: 25% needed a reintervention, 12% underwent pacemaker implantation and 9% had supraventricular arrhythmias. Early reinterventions were mainly for residual PS, late reinterventions for pulmonary regurgitation. Subjective health status was good. Exercise capacity was normal in 74% (median 96 (82–107)% of expected workload). Right ventricular and left ventricular (LV) dysfunction was found in 13% and 41%, respectively. The use of a transannular patch and younger age at surgery were predictive for late events (HR 3.02 (95% CI 1.09 to 8.37) and HR 0.81/year (95% CI 0.66 to 0.98), respectively). Use of inflow occlusion compared with cardiopulmonary bypass showed a trend towards more reinterventions (HR 3.19 (95% CI 0.97 to 10.47)).Conclusions Survival up to 40 years after successful PS repair is nearly normal. Subjective health status is good and there is a low incidence of arrhythmias. Reinterventions, however, are necessary in one-quarter and 40 years postoperatively several patients show LV dysfunction. ER -