title: Burden of Cardiac Siderosis in a Thalassemia-Major Endemic Population: A Preliminary Report From Pakistan.
abstract: To describe the initial experience and demographics of T2* cardiac magnetic resonance-based myocardial-iron quantification of transfusion-dependent thalassemia-major (TM) patients from Pakistan and the correlation with serum ferritin.
  
  
  
  Eligible TM patients presenting between April 2014 and April 2015 to Aga Khan University Hospital, Pakistan, for T2*CMR were included. The severity of myocardial-iron deposition was defined as follows: normal T2*>20 ms, mild-moderate T2*10 to 20 ms, and severe T2*<10 ms. Cardiac symptoms were classified using the NYHA functional classification. Echocardiographic systolic and diastolic functions were performed. Continuous variables were presented as the median (minimum-maximum value). Correlation was measured using the Spearman rank correlation. Multivariate logistic regression was used to determine factors associated with the NYHA functional class.
  
  
  
  A total of 83 patients (49 male and 34 female) with TM, age 19 (5 to 45) years at presentation for T2*CMR, were reviewed. At presentation, 70% of the patients were classified as NYHA class II or worse. T2*<20 ms was observed in 62.6% of the patients, with 47% showing severe iron deposition (T2<10 ms). No correlation of T2*<20 ms (r=-0.157, P=0.302) and T2*<10 ms (r=-0.128, P=0.464) was observed with serum ferritin. On multivariate analysis, lower T2* values correlated with a worsening NYHA functional class.
  
  
  
  There is a high prevalence of severe myocardial iron load in Pakistani TM patients. Serum ferritin did not correlate with T2* values. Lower T2* was the only clinical factor associated with the NYHA functional class.
authors.affiliation: Departments of *Pediatrics and Child Health †Medicine ‡Radiology §Aga Khan University Hospital ¶Oncology, Section of Haematology and Oncology #Pathology and Laboratory Medicine, Section of Haematology, Aga Khan University Hospital ∥Afzaal Memorial Thalassemia Foundation, Karachi, Pakistan **Cardiovascular Biomedical Research Unit, Royal Brompton and Harefield NHS Foundation Trust and Imperial College, London, UK.
authors.forename: Najveen
authors.initials: N
authors.lastname: Alvi
authors.forename: Fateh A
authors.initials: FA
authors.lastname: Tipoo
authors.forename: Ahmed
authors.initials: A
authors.lastname: Imran
authors.forename: Muhammad N
authors.initials: MN
authors.lastname: Ashraf
authors.forename: Asim
authors.initials: A
authors.lastname: Qidwai
authors.forename: Mohammed
authors.initials: M
authors.lastname: Khursheed
authors.forename: Bushra
authors.initials: B
authors.lastname: Moiz
authors.forename: Salman N
authors.initials: SN
authors.lastname: Adil
authors.forename: Zehra
authors.initials: Z
authors.lastname: Fadoo
authors.forename: Sadaf
authors.initials: S
authors.lastname: Altaf
authors.forename: Dudley J
authors.initials: DJ
authors.lastname: Pennell
authors.forename: Babar S
authors.initials: BS
authors.lastname: Hasan
mesh_terms: D000293:Adolescent; D000328:Adult; D009202:Cardiomyopathies / Q000000981:diagnostic imaging / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D019353:Endemic Diseases; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007501:Iron / Q000378:metabolism; D019190:Iron Overload; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010154:Pakistan; D012806:Siderosis / Q000000981:diagnostic imaging / Q000209:etiology*; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications* / Q000453:epidemiology
chemical_list: D005293:Ferritins; D007501:Iron