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

Session Rapid Fire - Cardiac multimodality imaging in systemic diseases

Speaker Antonella Meloni

Event : ESC Congress 2013

  • Topic : imaging
  • Sub-topic : Cardiac Magnetic Resonance (CMR)
  • Session type : Rapid Fire Abstracts

Authors : A Meloni (Pisa,IT), A Filosa (Napoli,IT), M Casale (Napoli,IT), L Gulino (Pisa,IT), E Chiodi (Ferrara,IT), P Keilberg (Pisa,IT), S Armari (Legnano,IT), V Positano (Pisa,IT), M Lombardi (Pisa,IT), A Pepe (Pisa,IT)

A. Meloni1 , A. Filosa2 , M. Casale2 , L. Gulino1 , E. Chiodi3 , P. Keilberg1 , S. Armari4 , V. Positano1 , M. Lombardi1 , A. Pepe1 , 1CMR Unit, Fondazione G.Monasterio CNR-Regione Toscana and Institute of Clinical Physiology - Pisa - Italy , 2UOC Pediatria - DH Talassemia AORNA. Cardarelli - Napoli - Italy , 3Servizio Radiologia Ospedaliera-Universitaria Arcispedale "S. Anna" di Ferrara - Ferrara - Italy , 4Reparto di Pediatria – Azienda Ospedaliera di Legnago U.O. - Legnano - Italy ,

Cardiovascular Magnetic Resonance (CMR)

European Heart Journal ( 2013 ) 34 ( Abstract Supplement ), 827

Purpose: 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 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, 13.6±3.4 years) enrolled in the MIOT 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 and in all the location 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.

Conclusion: In pediatric TM patients myocardial fibrosis is not a rare finding to keep in mind in the cardiological management. If appropriate treatment is 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

Global Heart T2* (ms)20.9±13.930.6±9.70.022
Left atrial area (cm2)18.3±3.115.9±3.90.050
LV end-diastolic volume index (ml/m2)102.9±23.587.0±16.30.005
LV stroke volume index (ml/m2)60.7±12.451.8±10.70.012
LV mass index (g/m2)65.3±11.453.8±11.40.003
RV stroke volume index (ml/m2)61.5±11.648.9±14.10.005

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