title: The unnatural history of the ventricular septal defect: outcome up to 40 years after surgical closure.
abstract: BACKGROUND
  Few prospective data are available regarding long-term outcomes after surgical closure of a ventricular septal defect (VSD).
  
  
  OBJECTIVE
  The objective of this study was to investigate clinical outcomes>30 years after surgical VSD closure.
  
  
  METHODS
  Patients who underwent surgical VSD closure during childhood between 1968 and 1980 were reexamined every 10 years. In 2012, we invited eligible patients to undergo another examination, which included electrocardiography, Holter monitoring, echocardiography, bicycle ergometry, measurement of N-terminal pro-B-type natriuretic peptide, and subjective health assessment.
  
  
  RESULTS
  Cumulative survival was 86% at 40 years. Causes of mortality were arrhythmia, heart failure, endocarditis, during valvular surgery, pulmonary hypertension, noncardiac causes, and unknown causes. Cumulative event-free survival after surgery was 72% at 40 years. Symptomatic arrhythmias occurred in 13% of patients and surgical or catheter-based reinterventions in 12%. Prevalence of impaired right ventricular systolic function increased from 1% in 2001 to 17% in 2012 (p=0.001). Left ventricular systolic function was impaired but stable in 21% of patients. Aortic regurgitation occurred more often in the last 20 years (p=0.039), and mean exercise capacity decreased (p=0.003). N-terminal pro-B-type natriuretic peptide (median: 11.6 pmol/l [interquartile range: 7.0 to 19.8 pmol/l]) was elevated (>14 pmol/l) in 38% of patients. A concomitant cardiac lesion, for example, patent ductus arteriosus, and aortic cross-clamp time were determinants of late events (hazard ratio: 2.84 [95% confidence interval: 1.23 to 6.53] and hazard ratio: 1.47 per 10 min [95% confidence interval: 1.22 to 1.99], respectively). Patients rated their subjective health status significantly better than a reference population.
  
  
  CONCLUSIONS
  Survival up to 40 years after successful surgical VSD closure is slightly lower than in the general Dutch population. Morbidity is not negligible, especially in patients with a concomitant cardiac lesion.
authors.affiliation: Department of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Myrthe E
authors.initials: ME
authors.lastname: Menting
authors.affiliation: Department of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Judith A A E
authors.initials: JA
authors.lastname: Cuypers
authors.affiliation: Department of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Petra
authors.initials: P
authors.lastname: Opić
authors.affiliation: Department of Child and Adolescent Psychiatry and Psychology, Sophia Children's Hospital, Rotterdam, the Netherlands.
authors.forename: Elisabeth M W J
authors.initials: EM
authors.lastname: Utens
authors.affiliation: Department of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Maarten
authors.initials: M
authors.lastname: Witsenburg
authors.affiliation: Department of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Annemien E
authors.initials: AE
authors.lastname: van den Bosch
authors.affiliation: Department of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Ron T
authors.initials: RT
authors.lastname: van Domburg
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
authors.forename: Folkert J
authors.initials: FJ
authors.lastname: Meijboom
authors.affiliation: Department of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Eric
authors.initials: E
authors.lastname: Boersma
authors.affiliation: Department of Cardiothoracic Surgery, Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: Ad J J C
authors.initials: AJ
authors.lastname: Bogers
authors.affiliation: Department of Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands. Electronic address: j.roos@erasmusmc.nl.
authors.forename: Jolien W
authors.initials: JW
authors.lastname: Roos-Hesselink
mesh_terms: D000328:Adult; D001022:Aortic Valve Insufficiency / Q000453:epidemiology; D001145:Arrhythmias, Cardiac / Q000453:epidemiology / Q000601:surgery; D004452:Echocardiography; D017079:Exercise Tolerance; D005260:Female; D006304:Health Status; D006345:Heart Septal Defects, Ventricular / Q000401:mortality / Q000601:surgery*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D020097:Natriuretic Peptide, Brain / Q000097:blood; D063868:Patient Outcome Assessment; D010446:Peptide Fragments / Q000097:blood; D011446:Prospective Studies; D057566:Self Report; D018754:Ventricular Dysfunction / Q000453:epidemiology
chemical_list: D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain
keywords: morbidity; quality of life; subjective health status; survival; ventricular septal defect