Selvaratnam Veena, Bee Sun Lee, S. Jameela, K. Pau
{"title":"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","authors":"Selvaratnam Veena, Bee Sun Lee, S. Jameela, K. Pau","doi":"10.31762/ahj2231.0104","DOIUrl":null,"url":null,"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.","PeriodicalId":72307,"journal":{"name":"ASEAN heart journal : Official journal of the ASEAN Federation of Cardiology","volume":"43 2 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Giant Coronary Artery Aneurysm in a Severe Hemophilia A with Acute Coronary Syndrome: A Case Report and Brief Literature Review\",\"authors\":\"Selvaratnam Veena, Bee Sun Lee, S. Jameela, K. Pau\",\"doi\":\"10.31762/ahj2231.0104\",\"DOIUrl\":null,\"url\":null,\"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.\",\"PeriodicalId\":72307,\"journal\":{\"name\":\"ASEAN heart journal : Official journal of the ASEAN Federation of Cardiology\",\"volume\":\"43 2 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-10-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"ASEAN heart journal : Official journal of the ASEAN Federation of Cardiology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.31762/ahj2231.0104\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"ASEAN heart journal : Official journal of the ASEAN Federation of Cardiology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.31762/ahj2231.0104","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Giant Coronary Artery Aneurysm in a Severe Hemophilia A with Acute Coronary Syndrome: A Case Report and Brief Literature Review
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.