Original articles

Systemic disease and the heart

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

¹ MRI Laboratory, Institute of Clinical Physiology, "G Monasterio Foundation"/CNR, Pisa, Italy
² Pediatria per le Emopatie Ereditarie, G Di Cristina Hospital ARNAS, Palermo, Italy
³ Ematologia II con Talassemia, "V Cervello" Hospital, Palermo, Italy
⁴ Pediatria II per le Emopatie Ereditarie, Villa Sofia-CTO Hospital, Palermo, Italy
⁵ Centro per la Cura delle Microcitemie, Cardarelli Hospital, Napoli, Italy
⁶ Centro Microcitemia ed Anemie Congenite, Galliera Hospital, Genova, Italy
⁷ Struttura Complessa di Cardiologia, Galliera Hospital, Genova, Italy
⁸ Epidemiology and Biostatistics Unit, Institute of Clinical Physiology, CNR, Pisa, Italy
⁹ Department 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

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; ² = 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.

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Myocardial fibrosis in thalassaemia: recalling the past or telling the future?

John-Paul Carpenter, Sanjay K Prasad, and Dudley J Pennell
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This article has been cited by other articles:

• Carpenter, J.-P., Prasad, S. K, Pennell, D. J (2009). Myocardial fibrosis in thalassaemia: recalling the past or telling the future?. Heart 95: 1646-1647 [Full Text]