title: Second natural history study of congenital heart defects. Results of treatment of patients with pulmonary valvar stenosis.
abstract: BACKGROUND
  From 1958 to 1969, 592 patients (mostly children) with pulmonary stenosis were admitted to the First Natural History Study of Congenital Heart Defects (NHS-1) after cardiac catheterization. Most with gradients < 50 mm Hg were managed medically; most with gradients > or = 80 mm Hg had pulmonary valvotomy. Among these with gradients of 50-79 mm Hg, some were managed medically, and some were managed surgically. Most had a second cardiac catheterization at conclusion of NHS-1. More than 15 years had 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.
  
  
  RESULTS
  Of the original cohort of 592 patients, 580 were alive at completion of NHS-1. New data were obtained on 464 (78.4%) of the original cohort. Probability of 25-year survival was 95.7%. Probability of survival was less (80%) in a subgroup of patients entering NHS-1 > 12 years old with cardiomegaly. Less than 20% of patients managed medically during NHS-1 subsequently required a valvotomy. Only 4% of operated patients required a second operation. Bacterial endocarditis occurred rarely. Ninety-seven percent were in New York Heart Association class I. There was a small but higher-than-normal prevalence of serious arrhythmias. Most patients, whether medically or surgically managed, had low Doppler maximum gradients. Final clinical status was excellent or good in 83%.
  
  
  CONCLUSIONS
  Probability of survival is similar to that of the general population, and the vast majority of patients are asymptomatic. If valvotomy or valvuloplasty is required in a child, reoperation is rarely necessary. Patients with gradients < 25 mm Hg do not experience an increase in gradient. Patients with a gradient > or = 50 mm Hg should have valvotomy or valvuloplasty. Choice of management of patients with gradients of 40-49 mm Hg remains debatable.
authors.affiliation: Department of Pediatric Cardiology, Columbia-Presbyterian Medical Center-Babies Hospital, New York, NY 10032.
authors.forename: C J
authors.initials: CJ
authors.lastname: Hayes
authors.forename: W M
authors.initials: WM
authors.lastname: Gersony
authors.forename: D J
authors.initials: DJ
authors.lastname: Driscoll
authors.forename: J F
authors.initials: JF
authors.lastname: Keane
authors.forename: L
authors.initials: L
authors.lastname: Kidd
authors.forename: W M
authors.initials: WM
authors.lastname: O'Fallon
authors.forename: D R
authors.initials: DR
authors.lastname: Pieroni
authors.forename: R R
authors.initials: RR
authors.lastname: Wolfe
authors.forename: W H
authors.initials: WH
authors.lastname: Weidman
mesh_terms: D000328:Adult; D000368:Aged; D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000209:etiology; D002648:Child; D015331:Cohort Studies; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D016016:Proportional Hazards Models; D011665:Pulmonary Valve Insufficiency / Q000175:diagnosis / Q000209:etiology; D011666:Pulmonary Valve Stenosis / Q000150:complications / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D012086:Reoperation; D016019:Survival Analysis; D016896:Treatment Outcome