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The genetic basis of aortic disease. Marfan syndrome and beyond
1Department of Pediatrics, John Dempsey Hospital, University of Connecticut Health Center, Farmington, USA. tsipouras@nso1.uchc.edu
Cardiology Clinics
|December 10, 1999
Abstract:
The Marfan syndrome and related disorders are systemic disorders of connective tissue. Proximal aorta is usually dilated. The molecular basis of Marfan syndrome has been elucidated, thus allowing prenatal diagnosis. Life expectancy has markedly improved due to the widespread use of beta-adrenergic receptor inhibitors and improved surgical management of the aortic disease.