title: Early impairment of myocardial function in young patients with beta-thalassemia major.
abstract: OBJECTIVE
  One of the chief causes of death in patients with beta-thalassemia major (TM) remains heart failure due to iron overload. We investigated possible differences in myocardial function between a population of young asymptomatic patients with TM and healthy controls all of whom underwent an echocardiographic study, including tissue Doppler (TDI) and strain imaging (SI) analysis and cardiac magnetic resonance imaging (MRI).
  
  
  METHODS
  30 young asymptomatic patients with TM (16 taking deferoxamine and 14 taking deferiprone) and 30 healthy subjects underwent a cardiac MRI with T2* technique and an echocardiographic evaluation including systolic myocardial velocities (Sm), early (Em) and late (Am) diastolic velocities and systolic strain (S) at the level of basal segments of the lateral left ventricle (LV), interventricular septum (Septal) and lateral right ventricle (RV) wall. The differences in T2* values and echocardiographic parameters were also compared in patients with TM subgrouped according to iron chelation therapy.
  
  
  RESULTS
  The following TDI and SI measures were lower in patients than in controls: LV-Sm (P < 0.05), S-LV (P < 0.001), Septal-Sm (P < 0.05), Septal-Em (P < 0.001), S-Septal (P < 0.001), RV-Sm (P < 0.001), RV-Em (P < 0.001), RV-Em/Am (P < 0.05) and S-RV (P < 0.05). Myocardial function was better in the patients receiving deferiprone than those receiving deferoxamine. T2* values were higher in controls than in patients with TM and in those treated with deferiprone than those treated with deferoxamine. MRI data well correlated with SI parameters.
  
  
  CONCLUSIONS
  Study underlines that, even in a population of young, asymptomatic and well-chelated patients with TM, there is an impairment of myocardial function and that this condition could be easily detected by more advanced ultrasound techniques such as TDI and SI. The better indices of myocardial function in patients treated with deferiprone clearly needs confirmation from larger prospective studies.
authors.affiliation: Dipartimento di Scienze Cardiovascolari, Respiratorie e Morfologiche, Università degli Studi di Roma La Sapienza, Rome, Italy. damiano.magri@libero.it
authors.forename: Damiano
authors.initials: D
authors.lastname: Magrì
authors.forename: Susanna
authors.initials: S
authors.lastname: Sciomer
authors.forename: Francesco
authors.initials: F
authors.lastname: Fedele
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Gualdi
authors.forename: Emanuele
authors.initials: E
authors.lastname: Casciani
authors.forename: Pellegrina
authors.initials: P
authors.lastname: Pugliese
authors.forename: Anna
authors.initials: A
authors.lastname: Losardo
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Ferrazza
authors.forename: Eleonora
authors.initials: E
authors.lastname: Pasquazzi
authors.forename: Erika
authors.initials: E
authors.lastname: Schifano
authors.forename: Eleonora
authors.initials: E
authors.lastname: Mussino
authors.forename: Raffaele
authors.initials: R
authors.lastname: Quaglione
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Piccirillo
mesh_terms: D000328:Adult; D004452:Echocardiography; D005260:Female; D006321:Heart / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D017086:beta-Thalassemia / Q000503:physiopathology*