흉부 및 복부 대동맥 박리를 동반한 Marfan 씨 증후군 = A Case of Marfan`s Syndrome with Dissecting Aneurysm Involving Thoracic and Abdominal Aorta

다국어 초록 (Multilingual Abstract)

Marfan's syndrome is a hereditary disorder of connective tissue fibers, involving skeleton, eye and cardiovascular system. The complications of cardiovascular system are mital regurgitation, aortic dilatation resulting in aortic regurgitation and frequent dissection of the ascending aorta. The authors have experienced a rare case of Marfan's syndrome with dissecting aneurysm involving only the descending aorta.
A 32-year-old man was admitted to Hanyang University Hospital because of sudden onset of chest pain, radiating to epigastrium and left shoulder. He had characteristic Marfanoid features such as a slender body with sparsity of subcutaneous fat, arachnodactyly and disproportionate long extremities as well as family history. A filr of the chest showed elongated, tortuous contours of aortic arch and dilated descending aorta. Emergency echocardiography, CT scan of the chest with contrast enhancement and aortography were performed and the patient was diagnosed as Marfan's syndrome with dissecting aneurysm of descending aorta. Operation was done.