Journal Information
Vol. 30. Issue 9.
Pages 735-741 (September 2011)
Vol. 30. Issue 9.
Pages 735-741 (September 2011)
Caso Clínico
Open Access
Imagens em Cardiologia… mas depois da clínica em Cardiologia… – Dissecção da aorta num doente com Síndrome de Marfan
Images in Cardiology after clinical observation – Aortic dissection in Marfan syndrome
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Nuno Cabanelasa,
Corresponding author
ncabanelas@gmail.com

Autor para correspondência.
, Ângelo Nobreb, Nuno Guerrab, Javier Gallegob, Ricardo Ferreirab, Catarina Carvalheirob, João Roqueb, Marisa Peresa, Luís Siopac, Vítor Paulo Martinsa, Graça Silvaa, João Cravinob
a Serviço de Cardiologia, Hospital Distrital de Santarém, Santarém, Portugal
b Serviço de Cirurgia Cárdio-Torácica, Hospital Santa Maria, Lisboa, Portugal
c Serviço de Medicina IV, Hospital Distrital de Santarém, Santarém, Portugal
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Resumo
Introdução

A Dissecção da Aorta tipo A de Stanford é um fenómeno raro, com alta mortalidade a curto prazo e com manifestações clínicas que por vezes tornam o diagnóstico diferencial um processo moroso pela necessidade de realização de várias modalidades de exames complementares no Serviço de Urgência.

Objectivos

Através da alusão a um caso clínico, pretende-se realçar a importância do exame objectivo na abordagem inicial destes doentes e da rapidez do acesso a um centro cirúrgico. É feita uma breve revisão acerca do diagnóstico e tratamento da Dissecção da Aorta Ascendente, e das particularidades desta na Síndrome de Marfan.

Caso clínico

Homem de 33 anos, admitido no Serviço de Urgência de um Hospital Distrital com quadro de dor dorsal e pré-cordial associada a vómitos, com 20 horas de evolução. O exame objectivo inicial mostrou a presença de sopro sistólico no foco aórtico e anomalias morfológicas músculo-esqueléticas compatíveis com Síndrome de Marfan. Com a suspeita de Dissecção da Aorta, realiza-se imediatamente ecocardiograma transtorácico que mostrou exuberante flap intimal com origem na junção sino-tubular. É transferido para o Serviço de Cirurgia Cárdio-Torácica do hospital de referência onde foi tratado com cirurgia tipo Bentall.

Conclusão

No caso apresentado, uma primeira abordagem na qual se realizou um exame objectivo atento levantou a hipótese de o doente pertencer a um grupo de alto risco para Dissecção da Aorta, evitando exames complementares desnecessários e morosos. Este diagnóstico exige tratamento cirúrgico emergente, de modo que se torna imperiosa a existência de um contacto directo, em tempo real, entre quem faz o diagnóstico e o cirurgião, bem como protocolos de acesso imediato a um centro cirúrgico.

Palavras-chave:
Disseção da aorta ascendente
Síndrome de Marfan
Cirurgia de Bentall
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.

Keywords:
Ascending aortic dissection
Marfan syndrome
Bentall procedure
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