Giant Coronary Artery Aneurysm in a Severe Hemophilia A with Acute Coronary Syndrome: A Case Report and Brief Literature Review

Selvaratnam Veena, Bee Sun Lee, S. Jameela, K. Pau
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Abstract

The management of acute coronary syndromes (ACSs) among adult patients with severe hemophilia A remains a clinical challenge, due to the use of anti-platelet, anti-thrombotic agents and/or revascularization procedures in them. We report here a case of severe hemophilia A presented with acute non-ST elevation myocardial infarction (NSTEMI), with incidental finding of coronary artery aneurysms (CAA), requiring an open-heart surgery. He successfully underwent a combined coronary artery bypass grafting (CABG) and closure of right coronary artery giant aneurysm with factor VIII (FVIII) replacement therapy, without bleeding complications nor requiring blood product transfusion. His FVIII activity level was maintained above 100% during the operation and 72 hours post-operatively. Thereafter, the FVIII coverage was reduced to maintain a trough level above 50% for another 1-week before switching back to his prophylactic dose. No inhibitor was detected. He was put on dual anti-platelets for 6 months, in which was reduced to single anti-platelet until today with no occurrence of chest pain. This case highlighted the occurrence of NSTEMI as a consequence of underlying CAA and its management challenge during the open-heart surgery.
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严重血友病a伴急性冠状动脉综合征并发巨大冠状动脉动脉瘤一例报告及简要文献复习
严重A型血友病成年患者急性冠状动脉综合征(ACSs)的管理仍然是一个临床挑战,因为他们使用抗血小板、抗血栓药物和/或血运重建术。我们在此报告一例严重的血友病a,表现为急性非st段抬高型心肌梗死(NSTEMI),附带发现冠状动脉瘤(CAA),需要心内手术。他成功地接受了冠状动脉旁路移植术(CABG)和右冠状动脉巨动脉瘤封闭与因子VIII (FVIII)替代治疗,无出血并发症,也不需要输血。术中及术后72小时FVIII活性维持在100%以上。此后,在转回预防性剂量之前,将FVIII覆盖率降低至维持50%以上的低谷水平1周。未检测到抑制剂。双抗血小板治疗6个月后降为单抗血小板治疗,至今未出现胸痛。该病例强调了NSTEMI作为潜在CAA的结果及其在心内直视手术期间的管理挑战。
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