Clinical management of the cardiovascular complications of the Marfan syndrome.
The Marfan syndrome is an autosomal dominant disorder of connective tissue with manifestations throughout the body. Diagnosis is based on the cardinal effects on the skeletal, ocular, and cardiovascular systems. Long limbs and digits, thoracic cage deformity, dislocation of the ocular lens, mitral valve prolapse, and dilatation of the aortic root are typical signs. Before recent advances in management, valvular regurgitation and aortic dissection accounted for over 90 percent of deaths, often in the third through fifth decades of life. Echocardiography, introduced nearly 2 decades ago, and the recent enhancements of cardiovascular Doppler techniques have greatly improved the ability of identifying patients with Marfan syndrome and following their clinical courses. Cardiovascular surgery has progressed over the same period to provide Marfan patients relatively safe approaches to repair of the life-threatening complications, and surgery should be offered prophylactically. The ability to intervene effectively in the Marfan syndrome requires a clear understanding of its natural history throughout life. This review discusses the clinical course and current management of the cardiovascular complications of the Marfan syndrome in children, young adults, and women considering pregnancy. These approaches have relevance to valvular and aortic problems found in the general population.
Gerry
J L
JL
Cardiology Dept, St Tammany Parish Hospital, Covington, LA.
Morris
L
L
Pyeritz
R E
RE
D001014:Aortic Aneurysm / Q000209:etiology / Q000601:surgery*; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications*; D008945:Mitral Valve Prolapse / Q000209:etiology / Q000601:surgery*