title: Echocardiographic findings in children with Marfan syndrome.
abstract: BACKGROUND
  The typical cardiac manifestations of Marfan syndrome are aortic regurgitation with progressive dilatation of the aortic root, which may cause dissection and rupture of the ascending aorta, mitral valve prolapse and mitral valve regurgitation. In this study, we aimed to show echocardiographic findings in 11 patients with Marfan syndrome.
  
  
  METHODS
  Diagnosis of Marfan syndrome was based on the Ghent criteria. All patients had a full echocardiographic evaluation. During the evaluation, we investigated the presence of mitral valve prolapse, mitral valve regurgitation, tricuspid valve prolapse, dilatation of the aortic root, and aortic regurgitation.
  
  
  RESULTS
  Eleven patients were diagnosed as Marfan syndrome (seven male, four female, age 4-14 years). All had mitral valve prolapse (nine with mitral valve regurgitation). Among these 11 patients, seven had accompanying tricuspid valve prolapse, six had dilatation of the aortic root and two had aortic regurgitation.
  
  
  CONCLUSIONS
  Eleven patients in our clinic were diagnosed as Marfan syndrome since they had distinct characteristics of marfanoid phenotype. Echocardiographic evaluation of these patients showed marked heart valve involvement. In Marfan syndrome, it is known that the aortic valve is affected following mitral valve involvement. In our experience, aortic root dilatation is less common. However, particular attention should be given to following up aortic root status with noninvasive echocardiography to institute measures to prevent complications.
authors.affiliation: Kecioren Training and Research Hospital, Ankara, Turkey. pedkard@gmail.com
authors.forename: O
authors.initials: O
authors.lastname: Ozdemir
authors.forename: R
authors.initials: R
authors.lastname: Olgunturk
authors.forename: S
authors.initials: S
authors.lastname: Kula
authors.forename: F S
authors.initials: FS
authors.lastname: Tunaoglu
mesh_terms: D000293:Adolescent; D001014:Aortic Aneurysm / Q000000981:diagnostic imaging* / Q000209:etiology; D001022:Aortic Valve Insufficiency / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005260:Female; D006349:Heart Valve Diseases / Q000000981:diagnostic imaging* / Q000209:etiology; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis; D008944:Mitral Valve Insufficiency / Q000000981:diagnostic imaging; D008945:Mitral Valve Prolapse / Q000000981:diagnostic imaging; D011237:Predictive Value of Tests; D011379:Prognosis; D014263:Tricuspid Valve Prolapse / Q000000981:diagnostic imaging; D014421:Turkey