4.6 Article

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

Journal

BRITISH JOURNAL OF HAEMATOLOGY
Volume 164, Issue 4, Pages 579-585

Publisher

WILEY-BLACKWELL
DOI: 10.1111/bjh.12643

Keywords

thalassaemia major; heart; magnetic resonance; iron overload; paediatric

Categories

Funding

  1. Chiesi Farmaceutici and ApoPharma Inc.
  2. 'Ministero della Salute

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The age at which it is necessary to start Cardiovascular Magnetic Resonance (CMR) T2* screening in thalassaemia major (TM) is still uncertain. To clarify this point, we evaluated the prevalence of myocardial iron overload (MIO), function and fibrosis by CMR in TM patients younger than 10years. We retrospectively selected 35 TM patients enrolled in the Myocardial Iron Overload in Thalassaemia network. MIO was measured by T2* multislice multiecho technique. Biventricular function parameters were evaluated by cine images. To detect myocardial fibrosis, late gadolinium enhancement images were acquired. Patients' age ranged from 42 to 97years. All scans were performed without sedation. Nine patients showed no MIO, 22 patients had heterogeneous MIO with a T2* global value 20ms; two patients had heterogeneous MIO with a T2* global value <20ms and two patients showed homogeneous MIO. No patient showed myocardial fibrosis. Among the patients with heart T2*<20ms, the youngest was 6years old, none showed heart dysfunction and the iron transfused was <35g in all cases. Cardiac iron loading can occur much earlier than previously described. The first cardiac T2* assessment should be performed as early as feasible without sedation, especially if chelation is started late or if poor compliance is suspected.

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