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Role of different phenotypic groups of thalassemia major patients studied by CMR

Session Best Posters 7

Speaker Antonella Meloni

Event : ESC Congress 2018

  • Topic : heart failure
  • Sub-topic : Imaging
  • Session type : Best ePosters

Authors : L Pistoia (Pisa,IT), A Meloni (Pisa,IT), S Salvadori (Pisa,IT), S Renne (Lamezia Terme,IT), P Giuliano (Palermo,IT), P Caccamo (Palermo,IT), M Rocca (Crotone,IT), L Rigoli (Messina,IT), MP Smacchia (Roma,IT), L Maffei (Roma,IT), A Spiga (Cagliari,IT), V Spadola (Ragusa,IT), N Romano (Reggio Emilia,IT), D Maddaloni (Fabriano,IT), A Pepe (Pisa,IT)

Authors:
L. Pistoia1 , A. Meloni1 , S. Salvadori2 , S. Renne3 , P. Giuliano4 , P. Caccamo4 , M. Rocca5 , L. Rigoli6 , M.P. Smacchia7 , L. Maffei8 , A. Spiga9 , V. Spadola10 , N. Romano11 , D. Maddaloni12 , A. Pepe1 , 1Gabriele Monasterio Foundation - Pisa - Italy , 2Institute of Clinical Physiology, CNR - Pisa - Italy , 3P.O. “Giovanni Paolo II” - Lamezia Terme - Italy , 4Ospedale Civico - Palermo - Italy , 5P. O. ASL 5 - Crotone - Italy , 6U.O. Polyclinic G. Martino - Messina - Italy , 7Policlinico Umberto I - Roma - Italy , 8Ospedale “Sant'Eugenio” - Roma - Italy , 9P.O. Pediatrico Microcitemico “A.CAO” - Cagliari - Italy , 10Azienda Ospedaliera Civile - O.M.P.A. Ragusa - Ragusa - Italy , 11Santa Maria Nuova Hospital - Reggio Emilia - Italy , 12Ospedale “Engles Profili” - Fabriano - Italy ,

Topic(s):
Chronic Heart Failure – Diagnostic Methods: Imaging

Citation:
European Heart Journal ( 2018 ) 39 ( Supplement ), 1287

Introduction: Beta thalassemia major (β-TM) displays a great deal of phenotypic heterogeneity, not fully investigated in terms of cause-effect.

Aim: We aimed to detect if different phenotypic groups could be related to different levels of cardiac impairments, evaluated by cardiovascular magnetic resonance (CMR).

Methods: We studied retrospectively 671 β-TM patients (age 30.1 years, 52,9% females) enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network. Myocardial iron overload was assessed by using a multislice multiecho T2* approach. Cine sequences were obtained to quantify biventricular functional parameters.

Results: Three groups of patients were identified: heterozygotes (N=279), homozygotes β+ (N=154), homozygotes β° (N=238). No significant differences for sex, age and haemato-chemical parameters were found among the groups. Transfusional needs resulted significantly lower in the homozygous β+ patients than the heterozygous (34.7 U vs 38.0 U, P<0.05) and the homozygous β° patients (34.7 U vs 41.6, P<0.0001). After adjusting for the transfusional requirements, the homozygous β+ group versus the homozygous β° group showed higher global heart T2* values (32.4 ms vs 26.2 ms, P<0.01); the number of segments with T2*<20ms and the number of patients with a global heart T2* value<20ms were significantly lower in the homozygous β+ group when compared to the other groups (see Figure). Moreover, after adjusting for cardiac iron, the homozygous β+ group showed a lower number of patients with a pathological left ventricular ejection fraction (LVEF) than the heterozygous and homozygous β° groups (27.2% vs 24.2% vs 14.3% P<0.05) (see Figure).

Conclusions: The homozygous β+ TM patients showed less myocardial iron overload and a concordant lower frequency of systolic heart dysfunction and cardiac remodelling. These data support the knowledge of the different phenotypic groups in the clinical and instrumental management of β-TM patients.

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