Korean Circ J.  1998 Feb;28(2):291-295. 10.4070/kcj.1998.28.2.291.

A Case of Marfan Syndrome with Dissecting Aortic Aneurysm Involving Thoracic and Abdominal Aorta

• Oh JY
• Kim SK
• Yun SK
• Lee KH
• Shin WY
• Kim CH
• Choi TM
• Kwon YJ

• KMID: 2228925
• DOI: http://doi.org/10.4070/kcj.1998.28.2.291

Abstract

Marfan syndrome is an inheritable connective tissue with protean clinical manifestations involving the ocular, skeletal and cardiovascular system. It is well estabalished that a clinical hall marker and the major cause of morbidity in Marfan syndrome is aortic root dilatation and associated aortic dissection which begins just above the coronary ostia in ascending aorta. We report a rare case of Marfan syndrome with aortic dissection which began just below left subclavian artery in descending aorta. A 20-years old woman was admitted to Soonchunhyang hospital because of sudden onset of back pain. On phsical examination, she had characteristic Marfanoid feature. Chest X-ray and Echocardiography showed cardiomegaly and severe dilatation of ascending aorta. Chest CT and aortography showed severe aneurysm of ascending aorta and aortic dissection of descending aorta with intimal flap. she was treated with medical treatment because of poor general condition.

Keyword

Marfan syndrome; Aortic aneurysm; Aortic dissection

MeSH Terms

Aneurysm
Aorta
Aorta, Abdominal*
Aorta, Thoracic
Aortic Aneurysm*
Aortography
Back Pain
Cardiomegaly
Cardiovascular System
Connective Tissue
Dilatation
Echocardiography
Female
Humans
Marfan Syndrome*
Subclavian Artery
Thorax
Tomography, X-Ray Computed
Young Adult