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Systemic disease and the heart
Myocardial scarring by delayed enhancement cardiovascular magnetic resonance in thalassaemia major
  1. A Pepe1,
  2. V Positano1,
  3. M Capra2,
  4. A Maggio3,
  5. C L Pinto4,
  6. A Spasiano5,
  7. G Forni6,
  8. G Derchi7,
  9. B Favilli1,
  10. G Rossi8,
  11. E Cracolici9,
  12. M Midiri9,
  13. M Lombardi1
  1. 1
    MRI Laboratory, Institute of Clinical Physiology, “G Monasterio Foundation”/CNR, Pisa, Italy
  2. 2
    Pediatria per le Emopatie Ereditarie, G Di Cristina Hospital ARNAS, Palermo, Italy
  3. 3
    Ematologia II con Talassemia, “V Cervello” Hospital, Palermo, Italy
  4. 4
    Pediatria II per le Emopatie Ereditarie, Villa Sofia-CTO Hospital, Palermo, Italy
  5. 5
    Centro per la Cura delle Microcitemie, Cardarelli Hospital, Napoli, Italy
  6. 6
    Centro Microcitemia ed Anemie Congenite, Galliera Hospital, Genova, Italy
  7. 7
    Struttura Complessa di Cardiologia, Galliera Hospital, Genova, Italy
  8. 8
    Epidemiology and Biostatistics Unit, Institute of Clinical Physiology, CNR, Pisa, Italy
  9. 9
    Department of Radiology, University of Palermo, Palermo, Italy
  1. 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

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.

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Footnotes

  • 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.

  • Competing interests None.

  • Provenance and Peer review Not commissioned; externally peer reviewed.

  • See Editorial, p 1646

  • Ethics approval Approved by the institutional ethics committee of Pisa (study protocol no 34008).

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