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Myocardial iron overload in thalassemia major. How early to check?

  • 1,
  • 2,
  • 2,
  • 1,
  • 3,
  • 4,
  • 5,
  • 6,
  • 1 and
  • 1
Journal of Cardiovascular Magnetic Resonance201416 (Suppl 1) :P254

https://doi.org/10.1186/1532-429X-16-S1-P254

  • Published:

Keywords

  • Cardiovascular Magnetic Resonance
  • Late Gadolinium Enhancement
  • Thalassemia
  • Myocardial Fibrosis
  • Deferoxamine

Background

It is still controversy in thalassemia major (TM) if Cardiovascular Magnetic Resonance (CMR) T2* screening should be initiated before the 10 years. To answer this question, we studied retrospectively the prevalence of cardiac iron and function and myocardial fibrosis by CMR in a consistent cohort of TM patients younger than 10 years.

Methods

From the 2171 patients enrolled in the MIOT (Myocardial Iron Overload in Thalassemia) network, we retrospectively selected the 35 TM patients aged less than 10 years who had undergone at least one MRI scan. Myocardial iron overload (MIO) was measured by T2* multislice multiecho technique. Biventricular function parameters were quantitatively evaluated in a standard way by cine images. To detect myocardial fibrosis, late gadolinium enhancement images were acquired.

Results

Patients' age ranged from 4.2 to 9.7 years. All MRI scans were performed without sedation. Nine patients (25.7%) showed no myocardial iron overload (MIO), 22 patients (62.9%) showed an heterogeneous MIO with a T2* global value ≥ 20 ms; 2 patients (5.7%) showed an heterogeneous MIO and a T2* global value < 20 ms and 2 patients (5.7%) had a homogeneous MIO (Figure 1). Biventricular function parameters were assessed only in 28/35 patients (80%), because for 7 patients a short MRI protocol was chosen to avoid sedation. LV dysfunction (EF < 54%) was found in one patient (male, 7-year old, treated with deferoxamine and showing an heterogeneous myocardial iron overload with a global T2* value = 31.1 ms). No patient showed RV dysfunction. Finally, 14 patients completed the MRI protocol with acquisition of the LGE images and none of them showed myocardial fibrosis. Table 1 reports the data of the 4 patients (3 males and 1 female) with significant myocardial iron overload (global heart T2* < 20 ms). The youngest patient was 6 years old, all patient showed no heart dysfunction and in all the iron transfused was less than 35 g.
Figure 1
Figure 1

Up: Representative bull's eye maps identifying the 4 patterns of myocardial iron overload (MIO). The pie chart specifies the percentage of patients for each pattern. Bottom: Bull's-eye representation of the 16 myocardial standard segments.

Table 1

Demographic, clinical and MRI data of the 4 patients with global heart T2* < 20 ms.

Parameter

Patient 1

Patient 2

Patient 3

Patient 4

Age (yrs)

9.5

6.8

8.8

7.9

Sex

M

F

M

M

Transfusions starting age (months)

12

7

12

12

Mean Hb pre-transfusion (g/dl)

9.0

9.7

9.8

9.6

Mean serum ferritin in the previous year (ng/ml)

4500

2488

2579

2359

Transfused iron (g)

32

14

23

27

Chelation starting age (months)

16

30

24

36

Chelation treatment at the time of MRI

Deferoxamine

Deferasirox

Deferasirox

Deferoxamine

Compliance

good

dubious

excellent

excellent

Previous chelation therapy

None

Deferoxamine

Deferoxamine

-Deferoxamine

-Deferasirox

Global heart T2*/Mid ventricular septum T2* (ms)

11.2/15

13/13

16.2/18

18.9/24.5

MRI CIC (mg/g dry weight)

2.35

1.97

1.51

1.25

N. of pathological segments

16

16

12

9

Pattern of MIO

Homogenous

Homogenous

Heterogeneous

Heterogeneous

MRI LIC (mg/g dry weight)

21.4

23.3

9.6

15.1

LV EF (%)

61

NE

63

59

RV EF (%)

63

NE

64

56

M = male, F = female; MRI = Magnetic Resonance Imaging; CIC = cardiac iron concentration; MIO = myocardial iron overload; LIC = liver iron concentration; LV = left ventricular; EF = ejection fraction; RV = right ventricular; NE = not evaluated.

Conclusions

The first cardiac T2* assessment should be performed as early as possible without sedation and it is mandatory whenever poor compliance is suspected or if chelation has been started late.

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)
Department of Clinical and Experimental Medicine (Pediatrics), University of Ferrara, Ferrara, Italy
(3)
UOSD Centro per le Microcitemie, AORN Cardarelli, Napoli, Italy
(4)
U.O.C. Ematologia con Talassemia ARNAS, Ospedale Civico, Palermo, Italy
(5)
Centro Talassemie ed Emoglobinopatie, Ospedale Meyer, Firenze, Italy
(6)
Servizio Radiologia Ospedaliera-Universitaria, Arcispedale "S. Anna" di Ferrara, Ferrara, 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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