Literature DB >> 15529906

Large ascending aortic aneurysm and severe aortic regurgitation in a 7-year-old child with Marfan syndrome and a review of the literature. Marfan syndrome in childhood.

Mehmet Yazici1, Serdar Soydinç, Vedat Davutoğlu, Ilyas Akdemir, M Hakan Dinçkal.   

Abstract

A 7-year-old girl was admitted because of dyspnea on exertion and palpitations. Her symptoms had gradually worsened for the last 6 months. She had physical features of the Marfan syndrome. Transthoracic echocardiography showed an ascending aortic aneurysm, severe aortic regurgitation, and mildly dilated left ventricle. Because of marked aortic aneurysm and severe aortic regurgitation, the patient was treated with a beta-blocker and an angiotensin converting enzyme inhibitor. Surgery was refused by her parents. We describe here a child with Marfan syndrome in whom significant dilatation of the ascending aorta and severe aortic regurgitation is encountered and major cardiovascular complications of Marfan syndrome were reviewed.

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Year:  2004        PMID: 15529906     DOI: 10.1023/b:caim.0000041934.86689.13

Source DB:  PubMed          Journal:  Int J Cardiovasc Imaging        ISSN: 1569-5794            Impact factor:   2.357


  24 in total

Review 1.  Valve-sparing aortic root replacement in patients with the Marfan syndrome.

Authors:  D Craig Miller
Journal:  J Thorac Cardiovasc Surg       Date:  2003-04       Impact factor: 5.209

2.  Revised diagnostic criteria for the Marfan syndrome.

Authors:  A De Paepe; R B Devereux; H C Dietz; R C Hennekam; R E Pyeritz
Journal:  Am J Med Genet       Date:  1996-04-24

3.  Effect of beta-adrenergic blockade on aortic root rate of dilation in the Marfan syndrome.

Authors:  M A Salim; B S Alpert; J C Ward; R E Pyeritz
Journal:  Am J Cardiol       Date:  1994-09-15       Impact factor: 2.778

4.  Replacement of the ascending aorta and aortic valve with a composite graft in patients with nondisplaced coronary ostia.

Authors:  J M Piehler; J R Pluth
Journal:  Ann Thorac Surg       Date:  1982-04       Impact factor: 4.330

5.  Phenotypic features and impact of beta blocker or calcium antagonist therapy on aortic lumen size in the Marfan syndrome.

Authors:  R Rossi-Foulkes; M J Roman; S E Rosen; R Kramer-Fox; K H Ehlers; J E O'Loughlin; J G Davis; R B Devereux
Journal:  Am J Cardiol       Date:  1999-05-01       Impact factor: 2.778

6.  Genotype and phenotype analysis of 171 patients referred for molecular study of the fibrillin-1 gene FBN1 because of suspected Marfan syndrome.

Authors:  B Loeys; L Nuytinck; I Delvaux; S De Bie; A De Paepe
Journal:  Arch Intern Med       Date:  2001-11-12

7.  Complete replacement of the ascending aorta with reimplantation of the coronary arteries: new surgical approach.

Authors:  C Cabrol; A Pavie; I Gandjbakhch; J P Villemot; G Guiraudon; L Laughlin; P Etievent; B Cham
Journal:  J Thorac Cardiovasc Surg       Date:  1981-02       Impact factor: 5.209

Review 8.  Mutations in the human gene for fibrillin-1 (FBN1) in the Marfan syndrome and related disorders.

Authors:  H C Dietz; R E Pyeritz
Journal:  Hum Mol Genet       Date:  1995       Impact factor: 6.150

9.  Life expectancy in British Marfan syndrome populations.

Authors:  J R Gray; A B Bridges; R R West; L McLeish; A G Stuart; J C Dean; M E Porteous; M Boxer; S J Davies
Journal:  Clin Genet       Date:  1998-08       Impact factor: 4.438

10.  Changes in aortic distensibility and pulse wave velocity assessed with magnetic resonance imaging following beta-blocker therapy in the Marfan syndrome.

Authors:  M Groenink; A de Roos; B J Mulder; J A Spaan; E E van der Wall
Journal:  Am J Cardiol       Date:  1998-07-15       Impact factor: 2.778

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  2 in total

1.  Heart rate, conduction and ultrasound abnormalities in adults with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type.

Authors:  Filippo Camerota; Marco Castori; Claudia Celletti; Marco Colotto; Silvia Amato; Alessandra Colella; Mario Curione; Chiara Danese
Journal:  Clin Rheumatol       Date:  2014-04-22       Impact factor: 2.980

2.  Isolated infrarenal abdominal aorta aneurysm in a 42-year-old patient with Marfan's syndrome: Case report.

Authors:  Hamid Jiber; Rita Hajji; Youssef Zrihni; Rachid Zaghloul; Othman Zizi; Abdellatif Bouarhroum
Journal:  SAGE Open Med Case Rep       Date:  2013-10-01
  2 in total

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