期刊
WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY
卷 8, 期 2, 页码 231-234出版社
SAGE PUBLICATIONS INC
DOI: 10.1177/2150135116629396
关键词
aortic arch; pulmonary arteries; vascular disease; genetics; CHD
Arterial tortuosity syndrome is a rare autosomal recessive connective tissue disease characterized by elongation, tortuosity, and aneurysmal formation of the large and middle-sized arteries sometimes associated with stenosis of the pulmonary arteries and/or aorta. We present three cases of severe arterial tortuosity with different manifestations. In two cases, the aortic arch was involved. Angiography showed a very tortuous aortic arch, with many loops and twists and a normal descending aorta. One required operation. The third case presented multiple severe stenoses of both pulmonary arteries with many tortuous segments. Surgical repair consisted of pulmonary artery augmentation utilizing a bovine pericardial patch from hilum to hilum.
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