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Diagnosis of CAPS relies on thrombosis of at least three different organs systems over 1 week, histopathological evidence of small vessel occlusion, and high aPL antibody titers. In a subset of precipitating circumstances, activation or disruption of endothelial cells in the microvasculature may occur along with cardiomyopathy. We present two cases of CAPS\u2010associated dilated cardiomyopathy at our institution, focusing on disease management, pathophysiology, and treatment. These patients were of Southeastern Asian descent, raising the possibility of genetic polymorphisms contributing to the development of cardiomyopathy. Both met CAPS criteria and both demonstrated clinicopathologic thrombotic microangiopathy (TMA) and complement activation and developed severe dilated cardiomyopathy with shock. Complement activation plays an important role in the development of CAPS and may be important in the pathogenesis of CAPS\u2010associated cardiomyopathy. Clinical suspicion for TMA as a pathophysiologic mechanism of unexplained heart failure in CAPS is important and increased awareness of cardiac side effects is necessary so that early treatment can be initiated to halt further cardiac and systemic complications.<\/jats:p>","DOI":"10.1002\/jha2.71","type":"journal-article","created":{"date-parts":[[2020,8,1]],"date-time":"2020-08-01T03:59:36Z","timestamp":1596254376000},"page":"44-50","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Acute dilated cardiomyopathy in the setting of catastrophic antiphospholipid syndrome and thrombotic microangiopathy: A case series and review"],"prefix":"10.1002","volume":"1","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8311-4326","authenticated-orcid":false,"given":"Melody","family":"Hermel","sequence":"first","affiliation":[{"name":"Department of Medicine Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Hermel","sequence":"additional","affiliation":[{"name":"Department of Medicine Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saif","family":"Azam","sequence":"additional","affiliation":[{"name":"Department of Medicine Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jerold","family":"Shinbane","sequence":"additional","affiliation":[{"name":"Department of Cardiology Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annahita","family":"Sarcon","sequence":"additional","affiliation":[{"name":"Department of Cardiology Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erika","family":"Jones","sequence":"additional","affiliation":[{"name":"Department of Cardiology Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arjun","family":"Mehta","sequence":"additional","affiliation":[{"name":"Department of Pathology Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luanda","family":"Grazette","sequence":"additional","affiliation":[{"name":"Department of Cardiology Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Howard","family":"Liebman","sequence":"additional","affiliation":[{"name":"Jane Anne Nohl Division of Hematology Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilene","family":"Weitz","sequence":"additional","affiliation":[{"name":"Jane Anne Nohl Division of Hematology Keck School of Medicine University of Southern California  Los Angeles California"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,7,31]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1515\/jpm-2017-0092"},{"issue":"3","key":"e_1_2_7_3_1","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1055\/s-0028-1082274","article-title":"Morbidity and mortality in the catastrophic antiphospholipid syndrome: pathophysiology, causes of death, and prognostic factors","volume":"34","author":"Espinosa G","year":"2008","journal-title":"Semin Thromb Hemost"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1080\/10245332.1996.11746296"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2015.1.53"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2003.014175"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.07.009"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-007-0634-x"},{"key":"e_1_2_7_9_1","article-title":"Catastrophic cerebral antiphospholipid syndrome presenting as cerebral infarction with haemorrhagic transformation after sudden withdrawal of warfarin in a patient with primary antiphospholipid syndrome","volume":"2010","author":"Wani AM","year":"2010","journal-title":"BMJ Case Rep"},{"issue":"1","key":"e_1_2_7_10_1","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1159\/000168432","article-title":"Postpartum hemolytic uremic syndrome associated with antiphospholipid antibodies. 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