Early impairment of myocardial function in young patients with beta-thalassemia major.
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.
Magrì
Damiano
D
Dipartimento di Scienze Cardiovascolari, Respiratorie e Morfologiche, Università degli Studi di Roma La Sapienza, Rome, Italy. damiano.magri@libero.it
Sciomer
Susanna
S
Fedele
Francesco
F
Gualdi
Gianfranco
G
Casciani
Emanuele
E
Pugliese
Pellegrina
P
Losardo
Anna
A
Ferrazza
Giancarlo
G
Pasquazzi
Eleonora
E
Schifano
Erika
E
Mussino
Eleonora
E
Quaglione
Raffaele
R
Piccirillo
Gianfranco
G
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*