Myocardial scarring by delayed enhancement cardiovascular magnetic resonance in thalassaemia major
- A Pepe1,
- V Positano1,
- M Capra2,
- A Maggio3,
- C L Pinto4,
- A Spasiano5,
- G Forni6,
- G Derchi7,
- B Favilli1,
- G Rossi8,
- E Cracolici9,
- M Midiri9,
- M Lombardi1
- 1MRI Laboratory, Institute of Clinical Physiology, “G Monasterio Foundation”/CNR, Pisa, Italy
- 2Pediatria per le Emopatie Ereditarie, G Di Cristina Hospital ARNAS, Palermo, Italy
- 3Ematologia II con Talassemia, “V Cervello” Hospital, Palermo, Italy
- 4Pediatria II per le Emopatie Ereditarie, Villa Sofia-CTO Hospital, Palermo, Italy
- 5Centro per la Cura delle Microcitemie, Cardarelli Hospital, Napoli, Italy
- 6Centro Microcitemia ed Anemie Congenite, Galliera Hospital, Genova, Italy
- 7Struttura Complessa di Cardiologia, Galliera Hospital, Genova, Italy
- 8Epidemiology and Biostatistics Unit, Institute of Clinical Physiology, CNR, Pisa, Italy
- 9Department of Radiology, University of Palermo, Palermo, Italy
- Correspondence to Dr A Pepe, MRI Laboratory, Institute of Clinical Physiology, CNR, and Gabriele Monasterio Foundation, Via Moruzzi 1, 56124 Pisa, Italy; alessia.pepe{at}ifc.cnr.it
- Accepted 13 January 2009
- Published Online First 1 June 2009
Abstract
Background: Cardiovascular magnetic resonance (CMR) by delayed enhancement (DE) enables visualisation of myocardial scarring, but no dedicated studies are available in thalassaemia major.
Objective: To investigate the prevalence, extent, clinical and instrumental correlates of myocardial fibrosis or necrosis by DE CMR in patients with thalassaemia major.
Patients: 115 Patients with thalassaemia major consecutively examined at an MRI laboratory.
Methods: DE images were acquired to quantify myocardial scarring. Myocardial iron overload was determined by multislice multiecho T2*. Cine images were obtained to evaluate biventricular function.
Results: DE areas were present in 28/115 patients (24%). The mean (SD) extent of DE was 3.9 (2.4)%. In 26 patients the location of fibrosis was not specific and patchy distribution was prevalent. Two patients showed transmural DE following coronary distribution. The DE group was significantly older than the no-DE group (31 (7.7) years vs 26 (7.7) years, p = 0.004). No significant relation with heart T2* values and biventricular function was found. A significant correlation was found between the presence of DE and changes in ECG (ECG abnormal in the DE group 22/28 patients and in the no-DE group 30/87 patients; χ2 = 14.9; p<0.001).
Conclusions: In patients with thalassaemia the significant presence of myocardial fibrosis/necrosis seems to be a time-dependent process correlating with cardiovascular risk factors and cardiac complications. Levels of HCV antibodies are significantly higher in the serum of patients with thalassaemia with myocardial fibrosis/necrosis. ECG changes showed a good accuracy in predicting myocardial scarring.
Footnotes
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Funding This study was supported by the Italian Foundation “Leonardo Giambrone” and is on behalf of the Society for Thalassemia and Hemoglobinopathies (SOSTE). AP was supported by a grant received from the “Centro per la lotta contro l’infarto” Onlus Fondation.
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Competing interests None.
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Provenance and Peer review Not commissioned; externally peer reviewed.
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See Editorial, p 1646
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Ethics approval Approved by the institutional ethics committee of Pisa (study protocol no 34008).









