Significant improvement of survival by T2* MRI in thalassemia major
Antonella Meloni1, Caterina Borgna-Pignatti2, Giovanni Carlo Del Vecchio3, Maria Antonietta Romeo4, Maria Rita Gamberini5, Federico Bonetti6, Maria Giovanna Neri1, Elisabetta Chiodi7, Vincenzo Positano1, and Alessia Pepe1

1Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy, 2Università di Ferrara, Ferrara, Italy, 3Uiversity of Bari, Bari, Italy, 4University of Catania, Catania, Italy, 5Arcispedale "S.Anna", Ferrara, Italy, 6Policlinic Foundation San Matteo IRCCS, Pavia, Italy, 7Arcispedale “S. Anna”, Ferrara, Italy

Synopsis

The introduction of T2* CMR for the reproducible and non-invasive assessment of myocardial iron overload reduced the likelihood of developing decompensated cardiac failure, allowing the reduction of cardiac mortality in chronically transfused TM patients

Introduction

In 2004 seven Italian centers reported survival data for patients with thalassemia major (TM) and showed that heart disease due to iron overload was the most common cause of death [1]. In the same years the accurate and noninvasive assessment of cardiac siderosis was made possible in Italy by the introduction of the T2* cardiovascular magnetic resonance (CMR) [2]. We aimed to evaluate if the deployment of T2* CMR had an impact on the mortality rate.

Methods

Four centers contributed to the present study, updating the data of the enrolled patients until August 31, 2010. For the patients who died, the date of the death represented the end of the study. 577 patients (264 females and 313 males) were included. All patients were born on or after January 1, 1960 and mean age at the follow-up was 28.04 ± 10.88 years (median 29.52 years). Patients had been diagnosed as being affected by TM at a mean age of 10.95 ± 16.05 months (median 7.49 months). Patients were uniformly treated. Mean transfusion starting age was 1.20 ± 1.59 years (median 0.8 years).

Results

One-hundred and fifty-nine (27.6%) patients died, 124 of whom (77.9%) died before the year 2000. Dead patients were significantly younger and they were more frequently males. Dead patients started chelation therapy significantly later. Dead patients showed an higher frequency of HIV, arrhythmias and heart failure (see Table). According to the Cox model, the following variables were identified as significant univariate prognosticators for the death: male sex (HR=1.87, 95%CI=1.34-2.60, P<0.0001), HIV (HR=2.55, 95%CI=1.25-5.20, P=0.010) and heart failure (HR=8.86, 95%CI=6.37-12.31, P<0.0001). MRI was not performed in 406 patients (70.4%) and no patient had been scanned before his/her death. Among the survivors, MRI was not performed in the 59% of the cases (P<0.0001). The absence of an MRI scan was a significant univariate prognosticator for death (HR=43.25, 95%CI=11.32-165.33, P<0.0001). The study was restricted to the patients dead after 2004 (19/159=12%) or followed until August 2010 (N=357). In this subgroup of 376 patients, MRI was not performed in the 52.4% of the survivors and in all dead patients (P<0.0001). The absence of a MRI exam was reconfirmed as a strong predictive factor for death (HR=49.37, 95%CI=1.08-2263.24, P=0.046). The Kaplan–Meier curve is showed in the Figure. The log-rank test revealed a significant difference in the curves (P<0.0001).

Conclusions

Our data suggests that the use of T2* CMR, that enables individually tailored chelation regimes reducing the likelihood of developing decompensated cardiac failure, allowed the reduction of cardiac mortality in chronically transfused TM patients.

Acknowledgements

We thank all the colleagues of the MIOT Network (https://miot.ftgm.it).

The MIOT project receives “no-profit support” from industrial sponsorships (Chiesi Farmaceutici S.p.A. and ApoPharma Inc.).

References

[1] Borgna et al. Haematologica 2004;89:1187-93.

[2] Pepe A et al. JMRI 2006;23:662-8.

Figures

Table 1. Comparison between dead patients and survivors.

Figure 1. Kaplan–Meier survival curve for patients dead after 2004 or followed until August 2010.



Proc. Intl. Soc. Mag. Reson. Med. 24 (2016)
0143