title: Clinical cardiac involvement in thrombotic thrombocytopenic purpura: a systematic review.
abstract: BACKGROUND
  Autopsy studies consistently demonstrate cardiac involvement in thrombotic thrombocytopenic purpura (TTP), but clinical evidence for cardiac abnormalities is rarely reported.
  
  
  METHODS
  This systematic review addresses the apparent discrepancy between autopsy and clinical data. English language articles were identified by keywords for both TTP and for cardiac symptoms, testing, or events. Patients were analyzed if they were more than 10 years old with idiopathic TTP.
  
  
  RESULTS
  Thirty articles were identified that described 111 eligible patients: 20 case reports described 27 patients, 9 retrospective cohort studies described 74 patients, and 1 prospective cohort study described 10 patients. Cardiac events included infarction (26 patients), congestive failure (17), arrhythmias (10), cardiogenic shock (6), and sudden cardiac death (8). Mortality was assessed in 101 patients: 55 died, and 48 autopsies were described. All demonstrated cardiac microvascular thrombi, hemorrhage, and/or necrosis. Follow-up information was reported in only 6 of the 16 patients who survived a cardiac event (follow-up duration, 10 days-2 years; median, 7 weeks).
  
  
  CONCLUSIONS
  The frequency and sequelae of clinical cardiac abnormalities in TTP cannot be accurately assessed because most patients were described in reports of few selected patients; many patients were reported before the availability of effective treatment for TTP and sensitive tests for cardiac involvement. Continuing case reports and cohort studies, however, suggest that cardiac abnormalities may be important and often unrecognized causes of mortality and morbidity in patients with TTP. Prospective studies are needed to determine if cardiac therapy can improve survival and long-term outcomes of patients with TTP.
authors.affiliation: Department of Medicine, College of Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, OK 73190, USA.
authors.forename: Beau M
authors.initials: BM
authors.lastname: Hawkins
authors.forename: Mazen
authors.initials: M
authors.lastname: Abu-Fadel
authors.forename: Sara K
authors.initials: SK
authors.lastname: Vesely
authors.forename: James N
authors.initials: JN
authors.lastname: George
mesh_terms: D001344:Autopsy; D005500:Follow-Up Studies; D006331:Heart Diseases / Q000201:enzymology / Q000473:pathology*; D006801:Humans; D011697:Purpura, Thrombotic Thrombocytopenic / Q000473:pathology*