title: The unnatural history of pulmonary stenosis up to 40 years after surgical repair.
abstract: To provide prospective information on long-term outcome after surgical correction of valvular pulmonary stenosis (PS).
  
  
  
  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.
  
  
  
  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)).
  
  
  
  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.
authors: Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors: Judith A Ae
authors: JA
authors: Cuypers
authors: Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors: Myrthe E
authors: ME
authors: Menting
authors: Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors: Petra
authors: P
authors: Opić
authors: Department of Child and Adolescent Psychiatry/Psychology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors: Elisabeth M Wj
authors: EM
authors: Utens
authors: Department of Pediatrics, Division of Cardiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors: Willem A
authors: WA
authors: Helbing
authors: Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors: Maarten
authors: M
authors: Witsenburg
authors: Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors: Annemien E
authors: AE
authors: van den Bosch
authors: Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors: Ron T
authors: RT
authors: van Domburg
authors: Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors: Sara J
authors: SJ
authors: Baart
authors: Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors: Eric
authors: E
authors: Boersma
authors: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: Folkert J
authors: FJ
authors: Meijboom
authors: Department of Cardio-thoracic Surgery, Erasmus MC, Rotterdam, The Netherlands.
authors: Ad J Jc
authors: AJ
authors: Bogers
authors: Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
authors: Jolien W
authors: JW
authors: Roos-Hesselink
mesh_terms: D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D015415:Biomarkers / Q000097:blood; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D018572:Disease-Free Survival; D004452:Echocardiography; D015716:Electrocardiography, Ambulatory; D005080:Exercise Test; D017079:Exercise Tolerance; D005260:Female; D006304:Health Status; D006801:Humans; D053208:Kaplan-Meier Estimate; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D009426:Netherlands; D010446:Peptide Fragments / Q000097:blood; D016016:Proportional Hazards Models; D011446:Prospective Studies; D011664:Pulmonary Valve / Q000503:physiopathology / Q000601:surgery*; D011666:Pulmonary Valve Stenosis / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D020127:Recovery of Function; D019233:Retreatment; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D018497:Ventricular Dysfunction, Right / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right; D055815:Young Adult
chemical_list: D015415:Biomarkers; D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain