From 1958 through 1973. 2,339 patients (infancy through 21 years of age) with congenital aortic valve stenosis, pulmonary valve stenosis and ventricular septal defect were studied at 6 medical centers (Boston Children's Hospital, Buffalo Children's Hospital, University of Colorado Medical Center, Columbia Presbyterian Hospital, Johns Hopkins Hospital and Mayo Clinic). Some were treated medically and others had surgical management. All patients had cardiac catheterization on admission and most had a repeat catheterization 4 or 8 years following admission to the study. This was the largest study of patients with these lesions, and from the results the investigators defined the clinical course, indications for and timing of surgical management, and results of surgical management. However, only a small number of patients were adults at the time of the final examination and the follow-up period was relatively brief. Now, 10 years after completion of that study, most of these patients are adults, the oldest of whom are in their fifth decade of life. The proposed study will assess progression of severity of the defect, mortality rate, life experience, and long-term effects of medical or surgical management. This study will include a detailed report of each surviving patient's health status. Patients will return to one of the participating medical centers for history and physical examination and testing. The testing procedure will depend upon the type and severity of the defect. The tests that some, but not all, will have include electrocardiogram, chest x-ray, echocardiogram and Doppler, exercise test, 24-hour Holter monitoring, and hemoglobin and serum uric acid levels. It will allow us, in those patients who have cardiac catheterization, to refine the correlation of non-invasive methods of assessment with hemodynamic measurements. This study will be crucial in confirming or redefining the natural history of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect and the indications for and results of medical and surgical management. Data also will be available to add information on the relative contributions of cardiac output and systemic resistance to blood pressure in childhood, and """"""""tracking"""""""" of blood pressure from childhood into adulthood.

National Institute of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Research Project (R01)
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Epidemiology and Disease Control Subcommittee 3 (EDC)
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Mayo Clinic, Rochester
United States
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O'Fallon, W M; Rings, L; Gonzalez, R et al. (1987) Natural History Study of Congenital Heart Defects. Control Clin Trials 8:115S-120S