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 eventfree 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.

Additional Metadata
Persistent URL dx.doi.org/10.1136/heartjnl-2015-309159, hdl.handle.net/1765/96744
Journal Heart
Citation
Cuypers, J.A.A.E, Menting, M.E, Opic, P, Utens, E.M.W.J, Helbing, W.A, Witsenburg, M, … Roos-Hesselink, J.W. (2017). The unnatural history of pulmonary stenosis up to 40 years after surgical repair. Heart, 103(4), 273–279. doi:10.1136/heartjnl-2015-309159