Antonella Meloni1, Nicola Giunta2, Pietro Giuliano2, Maria Giovanna Neri1, Stefania Renne3, Antonino Vallone4, Massimiliano Missere5, Emanuele Grassedonio6, Vincenzo Positano1, Daniele De Marchi1, and Alessia Pepe1
1Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy, 2"ARNAS" Civico, Palermo, Italy, 3P.O. “Giovanni Paolo II”, Lamezia Terme (CZ), Italy, 4Az. Osp. "Garibaldi" Presidio Ospedaliero Nesima, Catania, Italy, 5P.O. “Giovanni Paolo II”, Campobasso, Italy, 6Policlinico "Paolo Giaccone", Palermo, Italy
Synopsis
We
prospectively assessed the predictive value of MRI for cardiac complications in talassemia intermedia (TI).
All our patients
underwent MRI examination assessing multi-parametric findings (heart and liver
iron, myocardial fibrosis, atrial areas, and biventricular function).
Righ ventricular (RV) hypertrophy identified patients at high risk for
arrhythmias and pulmonary hypertension. Both RV hypertrophy and fibrosis detected by LGE were
independent predictive factor for cardiac complications.
So, a widespread
program using MRI exploiting its multi-parametric potential, including the measurement of RV mass, can have
considerable power for opening the prognosis of TI patients by the early
identification and treatment of patients at risk for cardiac complications.
Introduction
Cardiovascular Magnetic Resonance (CMR) has an
established role in managing and predicting prognosis of patients with
Thalassemia Major (TM) [1]. Thalassemia Intermedia (TI) is a milder variant of thalassemia
showing a different clinical and prognostic profile; pulmonary hypertension
(PH) is a more common complication in TI patients [2].
We prospectively determined the predictive value
of CMR parameters, including measurement of right ventricular mass, for cardiac
complications in TI.
Methods
We considered 342 TI
patients enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) Network
[3]; about half of them (178/302, 58.9%) were transfusion-dependent.
Myocardial
and liver iron overload were measured by T2* multiecho technique [4]. Atrial
dimensions, left and right ventricular mass and systolic function were
quantified by cine images [5]. Late gadolinium enhancement (LGE) images were
acquired to detect myocardial fibrosis [6].
Results
Twenty-three
patients were excluded because a cardiac complication was present at the time
of first CMR, so we prospectively followed 319 patients. All 319 patients were
white, with a mean age at time of their first scan of 38.02±11.69 years and 165
(51.7%) of them were females.
Mean
follow-up time was 52.24±24.87 months (median 54.64 months).
Cardiac
events were recorded in 22 patients (6.9%): heart failure (HF) in 1 patient,
arrhythmias in 12 patients, pulmonary hypertension (PH) in 7 patients and
myocardial infarction (MI) in 2 patients.
Due to
the low number of events, only arrhythmias, PH and cardiac complications
globally considered were taken as cardiac outcomes for univariate and
multivariate analysis.
In the
multivariate analysis RV hypertrophy was the only independent predictive factor
for arrhythmias (HR=33.83, 95% CI=6.07-188.74, P<0.0001) and PH (HR=73.33,
95% CI=10.00-537.57, P<0.0001). When cardiac complications were considered
all together, RV hypertrophy (HR=24.12, 95% CI=5.09-114.12, P<0.0001) and
myocardial fibrosis by LGE (HR=6.59, 95% CI=1.33-32.67, P=0.021) were
independent prognostic factors in the multivariate analysis.
The Figures display the Kaplan–Meier curves showing
the impact of the independent predictive factors on each outcome.
Conclusions
For the first time we
studied the prognostic value of right ventricular mass as part of
multiparametric CMR imaging in a population of TI patients. RV hypertrophy
identified patients at high risk for arrhythmias and PH. Both RV hypertrophy
and fibrosis detected by LGE were independent predictive factor for cardiac
complications. Measurement of RV mass should be part of the multi-parametric
CMR study of patient with thalassemia intermedia.
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] Meloni A et al. Eur Heart J
2013;34(s1):292.
[2] Aessopos A et al.
Chest 2005;127:1523-30.
[3] Meloni A et
al. Int J Med Inform 2009;78:503-12.
[4] Pepe A et al. JMRI 2006;23:662-8.
[5] Marsella M et al. Haematologica
2011;96:515–20.
[6] Pepe A et al. Heart
2009;95:1688-93.