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Myocardial fibrosis is a predictor of adverse outcomes in thalassemia intermedia patients
Antonella Meloni1, Nicola Giunta2, Pietro Giuliano2, Stefania Renne3, Laura Pistoia1, Vincenzo Positano1, Calogera Gerardi4, Vincenzo Spadola5, Petra Keilberg1, and Alessia Pepe1

1Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy, 2"ARNAS" Civico, Di Cristina Benfratelli, Palermo, Italy, 3Presidio Ospedaliero “Giovanni Paolo II”, Lamezia Terme, Italy, 4Presidio Ospedaliero "Giovanni Paolo II" - Distretto AG2 di Sciacca, Sciacca, Italy, 5Azienda Ospedaliera Civile - O.M.P.A. Ragusa, Ragusa, Italy

Synopsis

Myocardial fibrosis detected by LGE has been confirmed as an independent predictor of CV complications in thalassemia intermedia patients. Our finding is consistent with a growing evidence that LGE has a strong prognostic impact in thalassemic patients, warranting a close clinical and instrumental follow-up.

Purpose

Myocardial fibrosis detected by late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is currently used to predict adverse cardiovascular events in different cardiomyopathies.1 However, its clinical implications in thalassemia intermedia (TI) have not been studied.

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) network2 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.3

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.

Acknowledgements

No acknowledgement found.

References

1. Assomull RG, Prasad SK, Lyne J, et al. Cardiovascular magnetic resonance, fibrosis, and prognosis in dilated cardiomyopathy. J Am Coll Cardiol. 2006;48(10):1977-1985.

2. Meloni A, Ramazzotti A, Positano V, et al. Evaluation of a web-based network for reproducible T2* MRI assessment of iron overload in thalassemia. Int J Med Inform. 2009;78(8):503-512.

3. Positano V, Pingitore A, Giorgetti A, et al. A fast and effective method to assess myocardial necrosis by means of contrast magnetic resonance imaging. J Cardiovasc Magn Reson. 2005;7(2):487-494.

Figures

Table 1. Results of univariate Cox analysis.

Figure 1. Kaplan–Meier curves showing the impact of myocardial fibrosis.

Proc. Intl. Soc. Mag. Reson. Med. 25 (2017)
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