title: Second natural history study of congenital heart defects. Results of treatment of patients with aortic valvar stenosis.
abstract: BACKGROUND
  From 1958 to 1969, 462 patients (mostly children) with aortic 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 aortic valvotomy. Of those with gradients of 50-79 mm Hg, some were managed medically, and some were managed surgically. Most had a second cardiac 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 (the Second Natural History Study [NHS-2]) addresses the long-term results of medical and surgical management.
  
  
  RESULTS
  Of the original cohort of 462 patients, 440 were alive at completion of NHS-1. New data were obtained on 371 (80.3%) of the original cohort. Probability of 25-year survival was 92.4% for those admitted with gradients < 50 mm Hg and 81.0% for those with admission gradients > or = 50 mm Hg. More than half of the cardiac deaths were sudden and unexpected. Forty percent of patients managed medically during NHS-1 subsequently required surgical management. Almost 40% of operated patients required a second operation. Three percent of the original cohort subsequently had bacterial endocarditis. There was a higher-than-normal prevalence of serious arrhythmias. Of NHS-2 full participants, 92.3% were in New York Heart Association functional class I. Most patients had low Doppler mean gradients. Clinically, 46.6% had aortic valve regurgitation. The final clinical status was excellent in 29.9%, good in 22.8%, fair in 28.6%, and poor in 18.7%.
  
  
  CONCLUSIONS
  Patients with gradients < 25 mm Hg can be followed medically; however, progressive stenosis can occur, and approximately 20% will require intervention. If the gradient is > or = 50 mm Hg, there is a risk of serious arrhythmias and, possibly, sudden death. If the gradient is > or = 80 mm Hg, intervention is clearly indicated; as techniques improve, delaying intervention in patients with gradients of 50-79 mm Hg may not be advantageous. Patients with gradients of 25-49 mm Hg can be followed medically with annual evaluation.
authors.affiliation: Department of Pediatric Cardiology, Children's Hospital, Boston, Mass.
authors.forename: J F
authors.initials: JF
authors.lastname: Keane
authors.forename: D J
authors.initials: DJ
authors.lastname: Driscoll
authors.forename: W M
authors.initials: WM
authors.lastname: Gersony
authors.forename: C J
authors.initials: CJ
authors.lastname: Hayes
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; D001024:Aortic Valve Stenosis / Q000150:complications / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015150:Echocardiography, Doppler; D015716:Electrocardiography, Ambulatory; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D013902:Radiography, Thoracic; D012086:Reoperation; D016019:Survival Analysis; D016896:Treatment Outcome