{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T18:41:13Z","timestamp":1774377673800,"version":"3.50.1"},"reference-count":8,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2020,3,12]],"date-time":"2020-03-12T00:00:00Z","timestamp":1583971200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,6,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Atypical haemolytic uraemic syndrome (aHUS) is a life-threatening, genetic disease of complement-mediated thrombotic microangiopathy that typically presents as anaemia, thrombocytopenia, and renal failure. Cardiomyopathy is seen in up to 10% of aHUS cases, but the aetiology is not well-understood.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>A 63-year-old man recently was diagnosed with a thrombotic microangiopathy most consistent with aHUS by renal biopsy after presentation with acute renal failure requiring haemodialysis. He was started on therapy with complement inhibitor, eculizumab. Six weeks after diagnosis, he presented with progressive dyspnoea on exertion and chest pain. An echocardiogram demonstrated an acute drop in left ventricular ejection fraction to 20\u201325% with global hypokinesis. Left heart catheterization showed moderate, non-obstructive coronary artery disease. Cardiac magnetic resonance imaging demonstrated diffuse myocardial oedema. Endomyocardial biopsy revealed an arteriole with obliterative changes and a few possible fragmented red blood cells suggestive of thrombotic microangiopathy. There was no biopsy evidence of immune complex deposition or myocarditis. He was treated for heart failure and was maintained on eculizumab. On repeat echocardiogram 3 months later, the patient had complete recovery of his ejection fraction (60\u201365%).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>In this report, we describe complete recovery of aHUS-associated heart failure with eculizumab therapy and demonstrate for the first time that the aetiology of aHUS-associated heart failure is likely an acute thrombotic microangiopathy involving small intramyocardial arterioles, as demonstrated by cardiac biopsy.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytaa050","type":"journal-article","created":{"date-parts":[[2020,3,12]],"date-time":"2020-03-12T14:27:57Z","timestamp":1584023277000},"page":"1-6","source":"Crossref","is-referenced-by-count":5,"title":["Atypical haemolytic uraemic syndrome: a case report of a rare cause of reversible cardiomyopathy"],"prefix":"10.1093","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0803-0429","authenticated-orcid":false,"given":"Courtney M","family":"Campbell","sequence":"first","affiliation":[{"name":"Division of Cardiovascular Medicine, Department of Internal Medicine, Davis Heart & Lung Research Institute, The Ohio State University Wexner Medical Center, 473 W 12th Ave, Suite 200, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7536-6806","authenticated-orcid":false,"given":"Clarissa","family":"Cassol","sequence":"first","affiliation":[{"name":"Department of Pathology, The Ohio State University Wexner Medical Center, 129 Hamilton Hall, 1645 Neil Ave, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8977-6910","authenticated-orcid":false,"given":"Spero R","family":"Cataland","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, The Ohio State University Wexner Medical Center, B302 Starling Loving Hall, 320 West 10th Ave, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5009-2909","authenticated-orcid":false,"given":"Rami","family":"Kahwash","sequence":"first","affiliation":[{"name":"Division of Cardiovascular Medicine, Department of Internal Medicine, Davis Heart & Lung Research Institute, The Ohio State University Wexner Medical Center, 473 W 12th Ave, Suite 200, Columbus, OH 43210, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2020,3,12]]},"reference":[{"key":"2020042405560691100_ytaa050-B1","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1056\/NEJMra0902814","article-title":"Atypical hemolytic-uremic syndrome","volume":"361","author":"Noris","year":"2009","journal-title":"N Engl J Med"},{"key":"2020042405560691100_ytaa050-B2","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1182\/blood-2005-10-007252","article-title":"Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome","volume":"108","author":"Caprioli","year":"2006","journal-title":"Blood"},{"key":"2020042405560691100_ytaa050-B3","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1016\/j.amjmed.2005.02.019","article-title":"Incidence and prognosis of acute heart failure in the thrombotic microangiopathies","volume":"118","author":"Gami","year":"2005","journal-title":"Am J Med"},{"key":"2020042405560691100_ytaa050-B4","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1532\/IJH97.E0713","article-title":"Dilated cardiomyopathy during the course of hemolytic uremic 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Nephrol"},{"key":"2020042405560691100_ytaa050-B8","first-page":"327980","article-title":"Acute systolic heart failure associated with complement-mediated hemolytic uremic syndrome","volume":"2015","author":"Vaughn","year":"2015","journal-title":"Case Rep Hematol"}],"container-title":["European Heart Journal - Case 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