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Cardiovascular findings of children with Marfan syndrome

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Author(s): Osman Yılmaz | Meki Bilici | Özben Ceylan | Selmin Karademir | Utku Arman Örün | Senem Özgür | Mahmut Keskin | Filiz Şenocak

Journal: Journal of Clinical and Experimental Investigations
ISSN 1309-8578

Volume: 3;
Issue: 2;
Start page: 199;
Date: 2012;
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Keywords: Marfan syndrome | aortic root dilatation | mitral valve prolapse

ABSTRACT
Objectives: The aim of our study is to investigate thefrequency of structural heart diseases in patients withMarfan syndrome (MS) and to reveal the importance ofclinical follow-up in MS.Materials and methods: Study population consisted of17 patients admitted to the Pediatric Cardiology departmentbetween January 2005 and March 2010 with thediagnosis of MS according to the Ghent criteria. Patientswere evaluated for the eye, genetic and the cardiovascularsystem abnormalities. Physical examination findings,echocardiographic, and radiological examinations of thepatients were evaluated retrospectively.Results: Of the 17 cases, 9 were girls and 8 were males,ages ranged from 1 month to 17 years (mean 9.7 years).There was a second degree of kinship between mothersand fathers in 5 patients. Respiratory distress, syncope,chest pain and palpitation were the most seen in thepresentation complaint of the patients. Skeletal findingsobserved in 13 patients, 4 patients had subluxation ofthe lens. Two patients had positive family history. Whencardiovascular findings were examined, there were aorticroot dilatation with mitral valve prolapse and/or tricuspidvalve prolapse in 8 patients, mitral valve prolapse in 3patients only aortic root dilatation in 3 patients and mitralvalve prolapse and tricuspid valve prolapse in 3 patients.Mean follow-up period was 2.6 years, aneurysm and ruptureof the aorta wasn’t observed during this period.Conclusion: In patients with Marfan syndrome, regularfollow-up and cardiological evaluation should be done becausesignificant structural heart diseases can be seenin these patients. J Clin Exp Invest 2012; 3(2): 199-201
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