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  • Open Access

Myocardial fibrosis by CMR LGE in a large cohrt of pediatric thalassemia major patients

  • 1,
  • 2,
  • 2,
  • 3,
  • 1,
  • 4,
  • 5,
  • 1,
  • 1 and
  • 1
Journal of Cardiovascular Magnetic Resonance201416 (Suppl 1) :P395

https://doi.org/10.1186/1532-429X-16-S1-P395

  • Published:

Keywords

  • Cardiovascular Magnetic Resonance
  • Late Gadolinium Enhancement
  • Left Ventricular Mass
  • Thalassemia
  • Myocardial Fibrosis

Background

Cardiovascular Magnetic Resonance (CMR) by late gadolinium enhancement (LGE) allows to detect myocardial fibrosis. Myocardial fibrosis was shown to be a relative common finding in large cohort of Italian thalassemia major (TM) patients mainly related to HCV infection, but specific studies involving only pediatric patients are not available. Our aim was to investigate the prevalence and clinical-instrumental correlates of myocardial fibrosis in pediatric TM patients.

Methods

We studied retrospectively 76 pediatric patients with TM (44 boys, 4.2 -17.9 years old, mean age 13.6 ± 3.4 years) enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) Network. All patients were well transfused and chelated since the early childhood.LGE images were acquired to detect myocardial fibrosis. Myocardial iron overload (MIO) was measured by T2* multislice multiecho technique. Biventricular function parameters were quantitatively evaluated by cine images.

Results

Myocardial fibrosis was detected in 12 (15.8%) patients. In all patients the location of the fibrosis was epi-mesocardial, with no ischemic pattern. The youngest patient showing myocardial fibrosis had 13 years of age. Table 1 shows the comparison between patients with and without myocardial fibrosis. A significant higher MIO was detected in patients with myocardial fibrosis. The left atrial area, all the left ventricular (LV) indexed volumes, the LV mass index and the bi-ventricular stroke volume indexes were significantly higher in the fibrosis group than in the no-fibrosis group.
Table 1

Clinical and instrumental correlates in the fibrosis and no-fibrosis group.

 

Fibrosis

group

(N = 12)

No-fibrosis

group

(N = 64)

P-value

Sex (M/F)

10/2

34/30

0.062

Age (years)

15.4 ± 1.8

13.3 ± 3.5

0.073

Transfusions starting age (years)

1.2 ± 0.9

1.3 ± 0.8

0.691

Chelation starting age (years)

3.1 ± 1.8

3.1 ± 2.3

0.705

HCV antibodies, N (%)

0

3 (4.8%)

0.437

Hb pre-transfusion (g/dl)

9.7 ± 0.3

9.5 ± 0.7

0.757

Ferritin levels (ng/l)

3012 ± 2167

2225 ± 1396

0.226

ALT (u/l)

41.6 ± 12.5

38.6 ± 32.6

0.268

AST (u/l)

46.6 ± 41.2

33.4 ± 25.9

0.207

Global Heart T2* (ms)

20.9 ± 13.9

30.6 ± 9.7

0.022

MRI CIC (mg/g dry weight)

2.0 ± 1.7

0.8 ± 0.6

0.022

Patients with global heart T2* < 20 ms, N (%)

7 (58.3)

12 (18.8)

0.008

N. of seg. with abnormal T2*

9.0 ± 7.0

3.8 ± 5.2

0.030

Left atrial area (cm2)

18.3 ± 3.1

15.9 ± 3.9

0.050

Right atrial area (cm2)

16.9 ± 4.3

14.9 ± 3.5

0.169

Left ventricular end-diastolic volume index (ml/m2)

102.9 ± 23.5

87.0 ± 16.3

0.005

Left ventricular end-systolic volume index (ml/m2)

42.0 ± 12.1

35.1 ± 8.9

0.022

Left ventricular stroke volume index (ml/m2)

60.7 ± 12.4

51.8 ± 10.7

0.012

Left ventricular mass index (g/m2)

65.3 ± 11.4

53.8 ± 11.4

0.003

Left ventricular ejection fraction (%)

59.2 ± 4.4

59.7 ± 5.9

0.368

Right ventricular end-diastolic volume index (ml/m2)

96.9 ± 25.6

81.6 ± 17.1

0.089

Right ventricular end-systolic volume index (ml/m2)

36.9 ± 13.7

32.3 ± 8.3

0.458

Right ventricular stroke volume index (ml/m2)

61.5 ± 11.6

48.9 ± 14.1

0.005

Right ventricular ejection fraction (%)

62.6 ± 4.4

60.2 ± 7.1

0.175

Conclusions

In pediatric TM patients myocardial fibrosis is not a rare finding to keep in mind in the cardiological management. When appropriate treatment has been administered since early childhood, CMR LGE can be postponed until 13 years of age. By the natural history of this large cohort of pediatric patients where HCV infection has been appropriately prevented, myocardial fibrosis seem to be associated with MIO and high cardiac output.

Funding

The MIOT project receives "no-profit support" from industrial sponsorships (Chiesi Farmaceutici S.p.A. and ApoPharma Inc.). This study was also supported by: "Ministero della Salute, fondi ex art. 12 D.Lgs. 502/92 e s.m.i., ricerca sanitaria finalizzata anno 2006" and "Fondazione L. Giambrone".

Authors’ Affiliations

(1)
CMR Unit, Fondazione G.Monasterio CNR-Regione Toscana and Institute of Clinical Physiology, Pisa, Italy
(2)
Centro per le Microcitemie, AORN Cardarelli, Napoli, Italy
(3)
Centro Microcitemico, U.O. di Pediatria e Neonatologia, Presidio Ospedaliero Locri - A.S.L. n. 9, Locri, Italy
(4)
Istituto di Radiologia, Az. Osp. "Garibaldi" Presidio Ospedaliero Nesima, Catania, Italy
(5)
Dipartimento di Radiologia, Azienda Ospedaliero-Universitaria Ospedali Riuniti "Umberto I-Lancisi-Salesi", Ancona, Italy

Copyright

© Meloni et al.; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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