Serial echocardiographic left ventricular ejection fraction measurements: A tool for detecting thalassemia major patients at risk of cardiac death |
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| Authors: | Aurelio Maggio Angela Vitrano Giuseppina Calvaruso Rita Barone Paolo Rigano Luigi Mancuso Liana Cuccia Marcello Capra Lorella Pitrolo Luciano Prossomariti Aldo Filosa Vincenzo Caruso Calogera Gerardi Saveria Campisi Paolo Cianciulli Androulla Elefteriou Michel Angastiniotis Hala Hamza Nipon Chattipakorn |
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| Institution: | 1. U.O.C. Ematologia II, AOR Villa Sofia-V. Cervello, Palermo, Italy;2. Dipartimento di Scienze Statistiche e Matematiche ‘S. Vianelli’, Università di Palermo, Italy;3. U.O.C. Medicina Trasfusionale , AOR Villa Sofia-V. Cervello, Palermo, Italy;4. Fondazione Franco e Piera Cutino, Palermo, Italy;5. U.O.C. Centro di Prevenzione Diagnosi e Cura della Talassemia, ARNAS Civico, Di Cristina, Benfratelli, Palermo, Italy;6. U.O.C. Pediatria II, AOR Villa Sofia-V. Cervello, Palermo, Italy;7. Centro Regionale per la Cura delle Microcitemie, A.O. Cardarelli, Napoli, Italy;8. U.O.C. Pediatria A.O. Cardarelli, Napoli, Italy;9. U.O.D. Talassemia, P.O.S. Luigi Currò, ARNAS Garibaldi, Catania, Italy;10. U.O.S. Talassemia, A.O.O.C.R., Sciacca, Italy;11. U.O.S. Centro Microcitemia, A.O. Umberto I, Siracusa, Italy;12. U.O.D. Talassemia, A.O.S. Eugenio, Roma, Italy;13. Centro Microcitemia A.O. Perrino, Brindisi, Italy;14. Thalassemia International Federation, Nicosia, Cyprus;15. Department of Pediatric Cardiology, Cairo University, Egypt;p. Department of Paediatric Haematology and Oncology, St. Bartholomew''s and The Royal London Hospitals NHS Trust, London, United Kingdom;q. Royal Brompton and Harefield NHS Trust, London, United Kingdom;r. Cardiac Electrophysiology Research and Training Center, Faculty of Medicine, Chiang Mai University, Thailand |
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| Abstract: | Cardiac damage remains a major cause of mortality among patients with thalassemia major. The detection of a lower cardiac magnetic resonance T2* (CMR-T2*) signal has been suggested as a powerful predictor of the subsequent development of heart failure. However, the lack of worldwide availability of CMR-T2* facilities prevents its widespread use for follow-up evaluations of cardiac function in thalassemia major patients, warranting the need to assess the utility of other possible procedures. In this setting, the determination of left ventricular ejection fraction (LVEF) offers an accurate and reproducible method for heart function evaluation. These findings suggest a reduction in LVEF ≥ 7%, over time, determined by 2-D echocardiography, may be considered a strong predictive tool for the detection of thalassemia major patients with increased risk of cardiac death. The reduction of LVEF ≥ 7% had higher (84.76%) predictive value. Finally, Kaplan–Meier survival curves of thalassemia major patients with LVEF ≥ 7% showed a statistically significant decreased probability of survival for heart disease (p = 0.0022).However, because of limitations related to the study design, such findings should be confirmed in a large long-term prospective clinical trial. |
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