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Cardiovascular manifestations and treatment in Marfan syndrome [Anatol J Cardiol]
Anatol J Cardiol. 2005; 5(1): 46-52

Cardiovascular manifestations and treatment in Marfan syndrome

Vedat Erentuğ1, Adil Polat1, Kaan Kırali2, Esat Akıncı1, Cevat Yakut1
1Koşuyolu Kalp Eğitim ve Araştırma Hastanesi Kalp ve Damar Cerrahisi Kliniği, İstanbul
2Cardiovascular Surgery Center, Kartal Koşuyolu Yüksek İhtisas Education and Research Hospital, Koşuyolu, İstanbul, Turkey

Marfan syndrome is a dominantly inherited connective tissue disease characterized by cardiovascular, skeletal and ocular manifestations which was firstly described by Antoine Marfan in 1896. The underlying disorder is a mutation, which impairs fibrillin synthesis and is associated with the FBN-1 gene on the 15th chromosome. Ghent Nosology is used for diagnosis. The progressive dilatation of the proximal aorta leading to dissection and rupture is the typical feature of the disease. Aortic aneurysm and aortic rupture are the lethal complications of the disorder. Increased life expectancy depends on the control and the prevention of the cardiovascular complications. The most frequent cardiovascular manifestation is the mitral valve involvement. The principal pathologic findings on the mitral valve are annular dilatation, fibromyxomatous changes of the leaflets and chordae, elongation and rupture of chordae and deposition of calcium. Prolapsus occurs in 80% of the cases. Elective surgery with optimal timing is associated with increased survival. In the follow-up of patients after surgery for aortic aneurysm and dissection, serial imaging studies in order to detect a new onset aneurysm or dissection on different sites of the aorta are essential.

Keywords: Marfan syndrome, cardiovascular involvement

Vedat Erentuğ, Adil Polat, Kaan Kırali, Esat Akıncı, Cevat Yakut. Cardiovascular manifestations and treatment in Marfan syndrome. Anatol J Cardiol. 2005; 5(1): 46-52

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