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A Fontan procedure has been performed on 29 patients for tricuspid valvular atresia. The age range was 8 months to 33 years (median 10 years), the pulmonary vascular resistance ranged from 1.8-6.1 units . m2 (mean 3.3 units . m2), and the mean pulmonary arterial pressure ranged from 13-45 mm Hg (mean 21 mm Hg). Twenty-nine previous operations had been performed in 23 patients. Fourteen other associated cardiopulmonary anomalies were present in 12 patients. There were four hospital deaths (13.8%), all in patients with complicating features. Among the last 22 consecutive patients who have undergone operation, one died (4.5%). Complete atrioventricular block necessitated pacemaker implantation in one patient. No late deaths occurred. Of the 19 patients followed 3 months or more from the time of operation, eight have no restriction of exercise capacity, nine have only mild restriction, and two have a poor result. The Fontan approach to tricuspid atresia has several theoretical advantages over previously used shunts or pulmonary artery banding, the operative mortality in patients who have suitable anatomy and hemodynamics is low, and the results have been good.

Summary: Although congenital heart disease is often associated with hereditary factors1,2,3, subvalvular membranous aortic stenosis has previously not been reported in a family. This communication reports the occurrence of aortic subvalvular membrance in two siblings.

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