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Genotypic groups as risk factors for cardiac magnetic resonance abnormalities and complications in thalassemia major: a large, multicentre study.
Pistoia, Laura; Meloni, Antonella; Ricchi, Paolo; Filosa, Aldo; Lisi, Roberto; Maggio, Aurelio; Rosso, Rosamaria; Messina, Giuseppe; Iacono, Nicola Dello; Cuccia, Liana; Campisi, Saveria; Missere, Massimiliano; Midiri, Massimo; Vallone, Antonino; Renne, Stefania; Schicchi, Nicolò; Righi, Riccardo; Mangione, Maurizio; Positano, Vincenzo; Pepe, Alessia.
Afiliación
  • Pistoia L; "G. Monasterio Foundation", National Research Council - Region of Tuscany, Pisa, Italy.
  • Meloni A; "G. Monasterio Foundation", National Research Council - Region of Tuscany, Pisa, Italy.
  • Ricchi P; "A. Cardarelli" Hospital, Naples, Italy.
  • Filosa A; "A. Cardarelli" Hospital, Naples, Italy.
  • Lisi R; "Garibaldi-Centro" Hospital, Catania, Italy.
  • Maggio A; "V. Cervello" Hospital, Palermo, Italy.
  • Rosso R; "Vittorio Emanuele" University Hospital, Catania, Italy.
  • Messina G; "Bianchi-Melacrino-Morelli" Hospital, Reggio Calabria, Italy.
  • Iacono ND; "Casa Sollievo della Sofferenza" Research Hospital, San Giovanni Rotondo (FG), Italy.
  • Cuccia L; "Civico-Benfratelli-Di Cristina" Hospital, Palermo, Italy.
  • Campisi S; "Umberto I" Hospital, Siracusa, Italy.
  • Missere M; "Giovanni Paolo II" Research Hospital, Campobasso, Italy.
  • Midiri M; "Policlinico Paolo Giaccone" University Hospital, Palermo.
  • Vallone A; "Garibaldi-Nesima" Hospital, Catania, Italy.
  • Renne S; "Giovanni Paolo II" Hospital, Lamezia Terme, Italy.
  • Schicchi N; "Umberto I-Lancisi-Salesi" University Hospital, Ancona, Italy.
  • Righi R; "Delta" Hospital, Lagosanto (FE), Italy.
  • Mangione M; "G. Monasterio Foundation", National Research Council - Region of Tuscany, Pisa, Italy.
  • Positano V; "G. Monasterio Foundation", National Research Council - Region of Tuscany, Pisa, Italy.
  • Pepe A; "G. Monasterio Foundation", National Research Council - Region of Tuscany, Pisa, Italy.
Blood Transfus ; 19(2): 168-176, 2021 03.
Article en En | MEDLINE | ID: mdl-33000750
BACKGROUND: The causes and effects of genotypic heterogeneity in beta-thalassemia major (ß-TM) have not been fully investigated. The aim of this multicentre study was to determine whether different genotype groups could predict the development of cardiovascular magnetic resonance abnormalities and cardiac complications. MATERIALS AND METHODS: We considered 708 ß-TM patients (373 females, age 30.05±9.47 years) consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network. Data were collected from birth to the first cardiac magnetic resonance scan. Myocardial iron overload was assessed using a T2* technique. Biventricular function was quantified by cine images. Macroscopic myocardial fibrosis was evaluated by a late gadolinium enhancement technique. RESULTS: Three groups of patients were identified: ß+ homozygotes (n=158), ß+/ß° heterozygotes (n=298) and ß° homozygotes (n=252). Compared to ß+ homozygotes, the other two groups showed a significantly higher risk of myocardial iron overload and left ventricular dysfunction. We recorded 90 (13.0%) cardiac events: 46 episodes of heart failures, 38 arrhythmias (33 supraventricular, 3 ventricular and 2 hypokinetic) and 6 cases of pulmonary hypertensions. ß° homozygotes showed a significantly higher risk than ß+ homozygotes of arrhythmias and cardiac complications considered globally. DISCUSSION: Different genotype groups predicted the development of myocardial iron overload, left ventricular dysfunction, arrhythmias and cardiac complications in ß-TM patients. These data support the importance of genotype knowledge in the management of ß-TM patients.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades Cardiovasculares / Talasemia beta Tipo de estudio: Clinical_trials / Etiology_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Female / Humans / Male Idioma: En Revista: Blood Transfus Año: 2021 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Italia
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedades Cardiovasculares / Talasemia beta Tipo de estudio: Clinical_trials / Etiology_studies / Prognostic_studies / Risk_factors_studies Límite: Adult / Female / Humans / Male Idioma: En Revista: Blood Transfus Año: 2021 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Italia