Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects

Circulation. 1993 Feb;87(2 Suppl):I38-51.


Background: From 1958 to 1969, 1,280 patients (mostly children) with ventricular septal defects (VSDs) were admitted to the First Natural History Study of Congenital Heart Defects (NHS-1) after cardiac catheterization. Most with small defects and Eisenmenger's syndrome were managed medically; most with large VSDs were managed surgically. Of those with moderate-size defects, some were managed medically, and some were managed surgically. Most had a second catheterization at the conclusion of NHS-1. More than 15 years have elapsed since NHS-1, and most of the cohort are adults. This report (Second Natural History Study) addresses the long-term results of medical and surgical management.

Methods and results: Of an original cohort of 1,280 patients, 1,099 were alive at completion of NHS-1. New data were obtained on 976 (76.3%) of the original cohort. Probability of 25-year survival was 87%, and admission severity was the best predictor of survival. Of the 860 patients managed medically during NHS-1, 245 subsequently required surgical closure of the VSD. Only 5.5% of patients who had surgical closure required a second operation. On follow-up, there was a higher-than-normal prevalence of serious arrhythmias. Bacterial endocarditis occurred rarely. Of patients with small VSDs, 94.1% were in New York Heart Association functional class I. With the exception of those with Eisenmenger's syndrome, most patients had a final clinical status that was excellent or good.

Conclusions: The majority of patients fared well. However, there was a higher-than-normal prevalence of serious arrhythmia and sudden death, including those with small VSDs.

MeSH terms

  • Adolescent
  • Adult
  • Aortic Valve Insufficiency / etiology
  • Arrhythmias, Cardiac / epidemiology
  • Arrhythmias, Cardiac / etiology
  • Child
  • Child, Preschool
  • Cohort Studies
  • Electrocardiography
  • Female
  • Follow-Up Studies
  • Heart Septal Defects, Ventricular / complications
  • Heart Septal Defects, Ventricular / diagnosis
  • Heart Septal Defects, Ventricular / mortality
  • Heart Septal Defects, Ventricular / therapy*
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology
  • Infant
  • Male
  • Odds Ratio
  • Prevalence
  • Prognosis
  • Proportional Hazards Models
  • Reoperation
  • Survival Analysis