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Multimodal endovascular management of a Jehovah's Witness patient with Hugues-Stovin syndrome presenting with ruptured pulmonary artery aneurysm and cardiopulmonary thromboembolism.

作者信息

Kably Issam M, Reveron Carolina

机构信息

Department of Radiology, Section of Vascular and Interventional Radiology, Jackson Memorial Hospital, University of Miami Miller School of Medicine, Miami, FL, USA

Department of Radiology, University of Miami Miller School of Medicine, Miami, FL, USA.

出版信息

Eur J Cardiothorac Surg. 2015 Apr;47(4):e158-61. doi: 10.1093/ejcts/ezu529. Epub 2015 Feb 5.

Abstract

Hughes-Stovin syndrome is a very rare disease with fewer than 40 cases reported in the literature. The disease is thought to be a variant of Behçet's disease that can manifest with massive haemoptysis due to ruptured pulmonary arterial aneurysms in the setting of deep venous thrombosis. This association might impose a therapeutic dilemma when anticoagulation has to be balanced with the excessive risk of bleeding, especially if the patient refuses any blood replacement, and is not a surgical candidate. We present a case of a 41-year-old Jehovah's Witness patient with Hughes-Stovin syndrome that presented to the emergency room with haemoptysis due to multiple ruptured pulmonary artery aneurysms. Her work-up showed concomitant cardio-venous thromboembolism and pulmonary infarction. She was successfully managed via a bloodless single-wall venous puncture, allowing endovascular treatment of bilateral pulmonary aneurysms with subsequent caval filtration. The course was uneventful, and the patient was discharged under immunosuppressant.

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