Literature DB >> 23275854

Marfan Syndrome: Correct diagnosis can save lives.

Nafisa Samir1, Wafa Al-Fannah, Thord Theodorson, Abdulaziz Al-Mahrezi.   

Abstract

Marfan syndrome is a heritable disorder of the connective tissue that affects many systems of the body. However, the most serious complication in patients with Marfan syndrome is progressive enlargement of the aortic root, which may lead to aortic dissection, rupture, or aortic regurgitation. Prevention of these life threatening complications is very important in the management of this condition. A 39-year-old Omani man presented with progressive shortness of breath and eventually underwent major but successful cardiac surgery. It is very important to recognise Marfan syndrome early as preventive actions are possible if the condition is diagnosed before complications occur.

Entities:  

Keywords:  Aortic aneurysm; Aortic dissection; Case report; Marfan syndrome; Marfanoid habitus; Oman

Year:  2012        PMID: 23275854      PMCID: PMC3524007     

Source DB:  PubMed          Journal:  Sultan Qaboos Univ Med J        ISSN: 2075-051X


  13 in total

Review 1.  Management of Marfan syndrome.

Authors:  John C S Dean
Journal:  Heart       Date:  2002-07       Impact factor: 5.994

Review 2.  Treatment of aortic disease in patients with Marfan syndrome.

Authors:  Dianna M Milewicz; Harry C Dietz; D Craig Miller
Journal:  Circulation       Date:  2005-03-22       Impact factor: 29.690

3.  Usefulness of enalapril versus propranolol or atenolol for prevention of aortic dilation in patients with the Marfan syndrome.

Authors:  Anji T Yetman; Renee A Bornemeier; Brian W McCrindle
Journal:  Am J Cardiol       Date:  2005-05-01       Impact factor: 2.778

Review 4.  Marfan's syndrome.

Authors:  Daniel P Judge; Harry C Dietz
Journal:  Lancet       Date:  2005-12-03       Impact factor: 79.321

5.  Guidelines for the diagnosis and management of Marfan syndrome.

Authors:  Lesley Ades
Journal:  Heart Lung Circ       Date:  2006-12-26       Impact factor: 2.975

6.  Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan's syndrome.

Authors:  J Shores; K R Berger; E A Murphy; R E Pyeritz
Journal:  N Engl J Med       Date:  1994-05-12       Impact factor: 91.245

Review 7.  The molecular genetics of Marfan syndrome and related disorders.

Authors:  P N Robinson; E Arteaga-Solis; C Baldock; G Collod-Béroud; P Booms; A De Paepe; H C Dietz; G Guo; P A Handford; D P Judge; C M Kielty; B Loeys; D M Milewicz; A Ney; F Ramirez; D P Reinhardt; K Tiedemann; P Whiteman; M Godfrey
Journal:  J Med Genet       Date:  2006-03-29       Impact factor: 6.318

8.  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

9.  Angiotensin II blockade and aortic-root dilation in Marfan's syndrome.

Authors:  Benjamin S Brooke; Jennifer P Habashi; Daniel P Judge; Nishant Patel; Bart Loeys; Harry C Dietz
Journal:  N Engl J Med       Date:  2008-06-26       Impact factor: 91.245

10.  Life expectancy in the Marfan syndrome.

Authors:  D I Silverman; K J Burton; J Gray; M S Bosner; N T Kouchoukos; M J Roman; M Boxer; R B Devereux; P Tsipouras
Journal:  Am J Cardiol       Date:  1995-01-15       Impact factor: 2.778

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