Sotos syndrome is an overgrowth disorder of unknown etiology associated with a high incidence of congenital heart defects. Of 60 patients with Sotos syndrome treated in our hospital, 6 had congenital heart defects. We describe a case of successful total cavopulmonary connection at 30 months of age in a patient having pulmonary atresia with intact ventricular septum and a patent ductus arteriosus who had walking disability. The postoperative course was uneventful.
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