Korean Circ J.  1990 Sep;20(3):432-440. 10.4070/kcj.1990.20.3.432.

A Case of Marfan Syndrome with Left Atrial Myxoma Confirmed by Echocardiography

Abstract

The Marfan syndrome, a rare disease causing a marked decrease in life expectancy by involving the skeletal, ocular, and cardiovascular systems, is known as a connective tissue disorder that is inherited autosomal dominant. The cardiovascular complications directly related to the cause of death are associated with more than 90% of the Marfan syndrome. Aortic aneurysm with rupture could occur because of dilatation of aorta due to defect of media. Aortic and mitral insufficiency, mitral valve prolapse, bacterial endocarditis, arrhythmia, and aneurysm of interatrial septum are also frequently observed. A few literatures were reported domestically pertaining to the Marfan syndrome thus far, and there are increased discoveries of cardiovascular complications of the syndrome with the application of echocardiogram. However, the Marfan syndrome with LA myxoma has never been reported both domestically and internationally(INDEX MEDICUS, 1966-1989). Therefore we hereby report a case of the Marfan syndrome with LA myxoma from the observation of a patient who was admitted to Eulji General Hospital at Taejeon i August of 1989.


MeSH Terms

Aneurysm
Aorta
Aortic Aneurysm
Arrhythmias, Cardiac
Cardiovascular System
Cause of Death
Connective Tissue
Daejeon
Dilatation
Echocardiography*
Endocarditis, Bacterial
Hospitals, General
Humans
Life Expectancy
Marfan Syndrome*
Mitral Valve Insufficiency
Mitral Valve Prolapse
Myxoma*
Rare Diseases
Rupture
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