title: Benign Cardiac Effects of Hemoglobin H Disease.
abstract: Hemoglobin H (HbH) disease is associated with iron overload, but whether this results in serious cardiac or vascular sequelae is unresolved.
  
  
  
  We identified 39 adult subjects (age 42 ± 12 years, 13 men) with HbH disease who had undergone echocardiography, 27 of whom had also undergone cardiac and liver magnetic resonance assessment of iron loading using T2*-weighted imaging.
  
  
  
  None of the subjects had a history of heart failure or arrhythmias. There were 13/39 subjects with a ferritin level within the sex-based normal range and only 4/39 had ferritin >1,000 ng/ml. Left ventricular (LV) and left atrial dilatation was common, but LV ejection fraction was normal (≥55%) in all subjects. Age was positively correlated with log ferritin in the 27 nontransfused subjects (r = 0.43) and was inversely correlated with the transmitral E wave and E/A ratio (r = -0.69 and r = -0.79, respectively), but no relation of log ferritin with E or E/A was evident. The peak tricuspid regurgitation velocity was normal in 24/29 subjects for whom this was obtained, and it was no more than mildly elevated in the other 5. None of the tested subjects had an abnormal cardiac T2* reading, but half had evidence of liver iron loading.
  
  
  
  No myocardial iron loading or serious cardiac or vascular sequelae were identified in this cohort with HbH disease.
authors.affiliation: Thalassaemia Service, Monash University and Monash Health, Clayton, Vic., Australia.
authors.forename: Claire
authors.initials: C
authors.lastname: Sheeran
authors.forename: Donald K
authors.initials: DK
authors.lastname: Bowden
authors.forename: Sant-Rayn
authors.initials: SR
authors.lastname: Pasricha
authors.forename: Ken
authors.initials: K
authors.lastname: Cheng
authors.forename: Giovanni
authors.initials: G
authors.lastname: Romanelli
authors.forename: Roger E
authors.initials: RE
authors.lastname: Peverill
mesh_terms: D004452:Echocardiography; D006801:Humans; D019190:Iron Overload; D009206:Myocardium; D016277:Ventricular Function, Left; D017085:alpha-Thalassemia