Retrospective analysis of cardiac manifestations of our patients with marfan syndrome

Year 2011, Volume: 16 Issue: 4, 248 - 252, 22.01.2013

Hakki Simsek Musa Sahin Hasan Ali Gumrukcuoglu

Abstract

Abstract. Marfan syndrome (MS)   is an autosomal dominant connective tissue disorder affecting mainly cardiovascular system, eyes and skeleton. However, the most serious complication in patients with MS is progressive aortic root dilatation, aortic dissection or regurgitation. We have reviewed all patients with MS in our hospital over a six year period to determine the symptoms, clinical aspects, treatment modalities and long term follow-up. The medical records of all patients with MS in Yuzuncu Yil University Department of Cardiology from January 2004 to May 2010 were reviewed. MS was defined by Ghent criteria.  Individuals without a family history of MS require major criteria in at least two different organ systems and involvement of a third organ. Individuals carrying an FBN1 mutation known to cause MS or cases with a positive family history require one major criterion and involvement of an additional organ to diagnosis of MS. Eleven patients have diagnosis of MS according to Ghent criteria. Patients with mean age of 37.5 years. In our patient group wasn’t a presence woman. Main complaint of patients was dispnea. Primary findings in physical examination were apical systolo-diastolic murmur, mediastinal enlargement at chest X-ray. Aortic root dilatation, aortic regurgitation was seen echocardiographically. Mean follow-up time was 3.8 years.  During follow-up six patients died. Main cause of die was aortic complication. Early detection and close monitoring of the MS are very important for prevent complications. MS patients should be followed closely especially in terms of cardiovascular complications.

 

Key words: Marfan syndrome, cardiac manifestation

Keywords

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