ISSN : 1301-5680
e-ISSN : 2149-8156
Turkish Journal of Thoracic and Cardiovascular Surgery     
The Role of Echocardiography in The Diagnosis and Follow-up of Marfan Syndrome
Ayşe ÖZERGİN, Fuat BİLGEN, Fikri YAPICI, Ergin EREN, Serap Aykut ATA, Azmi ÖZLER
İstanbul Göğüs Kalp ve Damar Cerrahisi Merkezi
Marfan Syndrome that involves heart, skleton, lung, central nervous system is an autosomal dominant disease. The diagnosis of Marfan Syndrome is difficult except the classic phenotype. The aim of this study is to show the importance of echocardiography in the diagnosis of Marfan Syndrome and its cardiac complications. The fatal complication of Marfan Syndrome is aortic dissection. Other cardiac lesions are aortic root dilatation, aortic regurgitation (AR), mitral valve prolapse (MVP), mitral regurgitation (MR), rupture of chordae, tricuspid valve prolapse. The study group as consisted of seven patients. Their age were between 7-44 (mean 22.9). Skeleton, heart and eyes were involved in 2 patients, the others had only heart and skeleton involvement. Aortic root dilatation and MVP was detected in every patient. Nevertheless, AR was present in 4 patient and MR was present in 6 patient. There ere neither aortic dissection nor rupture of chordae during the follow-up time. Tricuspid valve prolapse was present in 6 patients. Positive family history was evident in only one patient. Aortic root dilatation was prominent on chest X-ray in 2 patients. Aortic root size ranged from 2.6 to 6.6 cem (mean 4.8 cm). The follow-up time was 1 to 21 months (mean 1 months).

In concluson, echocardiography must be the first diagnostic method in the identification of fatal and nonfatal cardiac complications and Marfan Syndrome except classical Marfan’s phenotype, also in the timing of surgical management.

Viewed : 44352
Downloaded : 8340