Case ReportOpen Access

Acute Aortic Dissection and Medullary Ischemia in a Patient with Marfan syndrome

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DOI: 10.23958/ijirms/vol04-i07/663· Pages: 441 to 445· Vol. 4, No. 07, (2019)· Published: July 24, 2019
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Abstract

Marfan syndrome (MS) is an autosomal dominant inherited connective tissue disease. The systemic complications of this disorder occur due to osteo-articular, cardiovascular, and ophthalmologic alterations. The cardiovascular involvement of MS is characterized mainly by aortic root and arch changes, resulting in increased susceptibility for the development of aneurysms. In the present study, we describe a case of type B abdominal aortic dissection in a patient with MS followed by spinal cord ischemia that rapidly evolved to paraplegia. Although rare, medullary ischemia is associated with a poor prognosis, as noted in the present case, wherein the patient died.

Keywords

Genetic syndromecardiovascular surgeryacute aortic syndrome
Author details
Gustavo Henrique Belarmino Góes
Medical Student at Faculty of Medical Sciences, University of Pernambuco, Recife, Brazil. Cardiovascular Emergency Room of Pernambuco (PROCAPE / University of Pernambuco), Recife, Brazil
✉ Corresponding Author
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Eugenio Soares de Albuquerque
Echocardiographer and Preceptor of the Cardiology Medical Residency of the Cardiovascular Emergency Room of Pernambuco (PROCAPE / University of Pernambuco), Recife, Brazil
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Diana Patricia Lamprea Sepulveda
Physician and Chief of the Ward of Valvular Diseases, Preceptor and Vice-Coordinator of the Cardiology Medical Residency of the Cardiovascular Emergency Room of Pernambuco (PROCAPE / University of Pernambuco), Recife, Brazil
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José Maria Del Castillo
CEO of the School of Ultrasound of Pernambuco (Ecope); Voluntary Professor of Specialization in Echocardiography of the Cardiovascular Emergency Room of Pernambuco (PROCAPE / University of Pernambuco). Vice-President of Echocardiography of the Department of Cardiovascular Image of the Brazilian Society of Cardiology, Recife, Brazil
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José Breno de Sousa Filho
Founding Member of the Latin-American Society of Interventional Cardiology, Assistant Professor at the University of Pernambuco, and Head of the Hemodynamics Laboratory of the Cardiovascular Emergency Room of Pernambuco (PROCAPE / University of Pernambuco), Recife, Brazil
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Ricardo de Carvalho Lima
Associate Professor of Cardiology at the University of Pernambuco; Executive Director of the Cardiovascular Emergency Room of Pernambuco (PROCAPE / University of Pernambuco). Head of Cardiovascular Surgery Team of the Cardiovascular Emergency Room of Pernambuco, Recife, Brazil;
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Dário Celestino Sobral Filho
Associate Professor of Cardiology at the University of Pernambuco. Research Coordinator of the Cardiovascular Emergency Room of Pernambuco (PROCAPE / University of Pernambuco), Recife, Brazil. Fellow of the American College of Cardiology and the European Society of Cardiology
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