EHA Library - The official digital education library of European Hematology Association (EHA)

PREDICTIVE ROLE OF MYOCARDIAL FIBROSIS IN THALASSEMIA INTERMEDIA PATIENTS
Author(s): ,
Antonella Meloni
Affiliations:
CMR Unit,Fondazione G. Monasterio CNR-Regione Toscana,Pisa,Italy
,
Martino Cinquetti
Affiliations:
Dipartimento Cardiovascolare,Azienda Ospedaliero-Universitaria 'Ospedali Riuniti',Trieste,Italy
,
Nicola Giunta
Affiliations:
Unità Operativa Complessa di Radiologia,ARNAS Civico “Benfratelli-Di Cristina”,Palermo,Italy
,
Pietro Giuliano
Affiliations:
Unità Operativa Complessa di Radiologia,ARNAS Civico “Benfratelli-Di Cristina”,Palermo,Italy
,
Vincenzo Positano
Affiliations:
CMR Unit,Fondazione G. Monasterio CNR-Regione Toscana,Pisa,Italy
,
Annamaria Carrà
Affiliations:
Unità Operativa di Pediatria,Ospedale “G. Da Saliceto”,Piacenza,Italy
,
Nicola Dello Iacono
Affiliations:
Centro Microcitemia – Day Hospital Thalassemia Poliambulatorio “Giovanni Paolo II”,Ospedale Casa Sollievo della Sofferenza IRCCS,San Giovanni Rotondo (FG),Italy
,
Antonella Massa
Affiliations:
Servizio trasfusionale,Ospedale “Giovanni Paolo II”,Olbia,Italy
,
Gianfranco Sinagra
Affiliations:
Dipartimento Cardiovascolare,Azienda Ospedaliero-Universitaria 'Ospedali Riuniti',Trieste,Italy
Alessia Pepe
Affiliations:
CMR Unit,Fondazione G. Monasterio CNR-Regione Toscana,Pisa,Italy
(Abstract release date: 05/19/16) EHA Library. Meloni A. 06/09/16; 133032; E1483
Dr. Antonella Meloni
Dr. Antonella Meloni
Contributions
Abstract
Abstract: E1483

Type: Eposter Presentation

Background
Myocardial fibrosis detected by late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is currently used to predict adverse cardiovascular events in different cardiomyopathies. However, its clinical implications in thalassemia intermedia (TI) have not been studied.

Aims
The aim of this study was to assess the distribution and the predictive value of myocardial fibrosis for future events in TI.

Methods
We considered 218 white TI patients (37.82±11.00 years, 113 females) consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network and free of cardiac complications concomitant to CMR. LGE images were acquired to detect myocardial fibrosis and the extent of LGE areas was quantified using a semiautomatic, previously validated software.

Results
Myocardial fibrosis was detected in 46 patients (21.1%). LGE followed a subendocardial ischemic pattern typical of coronary artery disease in two patients. One patient showed both an ischemic and non ischemic pattern. Thirty-two patients had a single focus while 14 had at least two foci. The mean number of LGE segments per patient was 2.70±1.52. LGE involved the septal region in 76.1% of patients. The extent of LGE areas was 2.09±1.77% of the total left myocardial mass.  Twenthy-two (47.8%) patients showed fibrosis in the infero- or anteroseptal junction, 18 (39.1%) had a non ischemic no-junctional pattern (intra or subepicardial) and 6 (13.0) had both a junctional and no-junctional pattern.Mean follow-up was 56.76±23.17 months. We recorded 13 cardiac complications: 1 heart failure, 7 arrhythmias and 5 pulmonary hypertension (PH). Myocardial fibrosis was a significant prognosticator for arrhythmias, PH and cardiac complications globally considered (see Table). Number of segments with LGE and extent of myocardial fibrosis were comparable in patients who developed cardiac complications versus the patients who did not.

Conclusion
In TI patients, myocardial fibrosis is a predictor of adverse outcome.



Session topic: E-poster

Keyword(s): Magnetic resonance imaging, Prognosis, Thalassemia
Abstract: E1483

Type: Eposter Presentation

Background
Myocardial fibrosis detected by late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is currently used to predict adverse cardiovascular events in different cardiomyopathies. However, its clinical implications in thalassemia intermedia (TI) have not been studied.

Aims
The aim of this study was to assess the distribution and the predictive value of myocardial fibrosis for future events in TI.

Methods
We considered 218 white TI patients (37.82±11.00 years, 113 females) consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network and free of cardiac complications concomitant to CMR. LGE images were acquired to detect myocardial fibrosis and the extent of LGE areas was quantified using a semiautomatic, previously validated software.

Results
Myocardial fibrosis was detected in 46 patients (21.1%). LGE followed a subendocardial ischemic pattern typical of coronary artery disease in two patients. One patient showed both an ischemic and non ischemic pattern. Thirty-two patients had a single focus while 14 had at least two foci. The mean number of LGE segments per patient was 2.70±1.52. LGE involved the septal region in 76.1% of patients. The extent of LGE areas was 2.09±1.77% of the total left myocardial mass.  Twenthy-two (47.8%) patients showed fibrosis in the infero- or anteroseptal junction, 18 (39.1%) had a non ischemic no-junctional pattern (intra or subepicardial) and 6 (13.0) had both a junctional and no-junctional pattern.Mean follow-up was 56.76±23.17 months. We recorded 13 cardiac complications: 1 heart failure, 7 arrhythmias and 5 pulmonary hypertension (PH). Myocardial fibrosis was a significant prognosticator for arrhythmias, PH and cardiac complications globally considered (see Table). Number of segments with LGE and extent of myocardial fibrosis were comparable in patients who developed cardiac complications versus the patients who did not.

Conclusion
In TI patients, myocardial fibrosis is a predictor of adverse outcome.



Session topic: E-poster

Keyword(s): Magnetic resonance imaging, Prognosis, Thalassemia

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