title: Cardiac events and cardiac T2* in Egyptian children and young adults with beta-thalassemia major taking deferoxamine.
abstract: OBJECTIVE
  Cardiac events and death are not uncommon in adults with beta-thalassemia (b-TM) taking deferoxamine (DFO) monotherapy because of poor compliance and possibly the less effectiveness of DFO in controlling cardiac iron overload. We sought to assess compliance with DFO, the percentage of shift to other iron chelators, and the occurrence of cardiac siderosis, and cardiac events and death in b-TM patients on DFO monotherapy.
  
  
  METHODS
  Prospective, observational, 10-year follow-up of patients attending Ain Shams Thalassemia Unit, Cairo, Egypt.
  
  
  METHODS
  For all b-TM patients aged 2-18 years attending the unit during January 1998 and taking DFO, we recorded all cardiac events (whether fatal or not) during January 2008. All patients still on DFO monotherapy and with a normal EKG and not showing symptoms or signs suggestive of heart failure (HF) were evaluated for cardiac siderosis by T2*.
  
  
  RESULTS
  Of 412 patients, only 126 (31%) were still taking DFO monotherapy (only 43% of those were compliant), 136 were taking combined DFO and deferiprone (DFP), 72 were taking DFP and 32 were taking deferasirox (DFX). Twenty-one were lost to follow-up and 25 died (10 cardiac). Eight of ten cardiac deaths and 12 of 15 non-cardiac deaths were in the DFO monotherapy group. Those taking DFO monotherapy with no HF and left ventricular ejection fraction (LVEF) by T2* >56% had a median age of 19 years and 56% were males; cardiac T2* was less than 20 ms in 30 (22%), 10-20 ms in 20 (14.7%) and less than 10 ms in 10 (7.3%). LVEF ranged from 58%-76% (median 64%). Forty percent of T2* patients less than 10 ms were compliant with DFO.
  
  
  CONCLUSIONS
  Fifty-eight percent of patients on DFO monotherapy were noncompliant, but even compliance did not prevent severe cardiac siderosis and most cardiac events (whether fatal or not) that occurred in the DFO monotherapy group.
authors.affiliation: Zagazig University, Zagazig, Sharkia, Egypt. usamaelsafy@yahoo.com
authors.forename: Mohsen S
authors.initials: MS
authors.lastname: Elalfy
authors.forename: Iman A
authors.initials: IA
authors.lastname: Abdin
authors.forename: Usama R
authors.initials: UR
authors.lastname: El Safy
authors.forename: Ahmed S
authors.initials: AS
authors.lastname: Ibrahim
authors.forename: Fatma S
authors.initials: FS
authors.lastname: Ebeid
authors.forename: Doria S
authors.initials: DS
authors.lastname: Salem
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001565:Benzoates / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003643:Death; D000077588:Deferasirox; D000077543:Deferiprone; D003676:Deferoxamine / Q000009:adverse effects / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D004534:Egypt; D004562:Electrocardiography; D005260:Female; D005293:Ferritins / Q000097:blood; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011446:Prospective Studies; D011728:Pyridones / Q000627:therapeutic use; D012737:Sex Factors; D017262:Siderophores / Q000009:adverse effects / Q000627:therapeutic use*; D012806:Siderosis / Q000150:complications*; D014230:Triazoles / Q000627:therapeutic use; D017086:beta-Thalassemia / Q000150:complications* / Q000188:drug therapy* / Q000401:mortality
chemical_list: D001565:Benzoates; D011728:Pyridones; D017262:Siderophores; D014230:Triazoles; D000077543:Deferiprone; D005293:Ferritins; D003676:Deferoxamine; D000077588:Deferasirox