Literature DB >> 21958999

[Images in cardiology after clinical observation - aortic dissection in Marfan syndrome].

Nuno Cabanelas1, Angelo Nobre, Nuno Guerra, Javier Gallego, Ricardo Ferreira, Catarina Carvalheiro, João Roque, Marisa Peres, Luís Siopa, Vítor Paulo Martins, Graça Silva, João Cravino.   

Abstract

INTRODUCTION: Stanford type A aortic dissection is a rare phenomenon with high short-term mortality and clinical manifestations that can make differential diagnosis a lengthy process requiring several diagnostic examinations.
OBJECTIVES: Based on a case report, the aim is to highlight the importance of physical examination in the initial management of these patients and of rapid access to a surgical center. A brief review follows on the diagnosis and treatment of ascending aortic dissection, and its specific nature in Marfan syndrome. CASE REPORT: A 33-year-old man was admitted to the emergency department of a district hospital with chest and back pain associated with vomiting, 20 hours after symptom onset. Initial physical examination revealed an aortic systolic murmur and musculoskeletal morphological abnormalities compatible with Marfan syndrome. Given suspected aortic dissection, a transthoracic echocardiogram was immediately performed, which showed an extensive intimal flap originating at the sinotubular junction. He was transferred to the cardiothoracic surgery department of a referral hospital where he was treated by a Bentall procedure.
CONCLUSION: In this case, careful physical examination during initial assessment raised the suspicion that this patient was in a high-risk group for aortic dissection, thus avoiding unnecessary and lengthy exams. This diagnosis requires emergent surgical treatment, and so direct contact in real time between those making in the diagnosis and the surgeon is essential, as well as protocols governing immediate access to a surgical center.
Copyright © 2011 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

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Year:  2011        PMID: 21958999     DOI: 10.1016/S0870-2551(11)70018-X

Source DB:  PubMed          Journal:  Rev Port Cardiol        ISSN: 0870-2551            Impact factor:   1.374


  1 in total

1.  Genotype-phenotype analysis of F-helix mutations at the kinase domain of TGFBR2, including a type 2 Marfan syndrome familial study.

Authors:  Lin Zhang; Ling-Gen Gao; Ming Zhang; Xian-Liang Zhou
Journal:  Mol Vis       Date:  2012-01-11       Impact factor: 2.367

  1 in total

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