Asian cardiovascular & thoracic annals
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Asian Cardiovasc Thorac Ann · Dec 2003
Case ReportsTotal cavopulmonary connection in a bedridden patient with Sotos syndrome.
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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Asian Cardiovasc Thorac Ann · Dec 2003
Extensive aortic surgery in Marfan syndrome: 16-year experience.
The aim of this study was to evaluate the clinical outcome of surgical treatment in patients with Marfan syndrome. Between 1985 and November 2001, 33 patients with Marfan syndrome were operated for chronic aneurysm of the aortic root with involvement of the ascending aorta in 20 patients and type A dissection in 13 patients. The patients comprised 24 males and 9 females with a mean age of 31.9 +/- 9.7 years (range, 18 to 54 years). ⋯ Aortic aneurysm was a significant univariate adverse factor for late aortic complications. Aortic surgery can be performed in Marfan patients with low morbidity and mortality. Aggressive surgical intervention does not impair surgical outcome while it decreases reoperation risk.