Purpose
Despite a significant increase in longevity for sickle cell disease (SCD) patients in the last decades, the mortality rate from cardiovascular and pulmonary complications remains high1.
Cardiac magnetic resonance (CMR) is the non-invasive gold standard for the quantification of biventricular functional parameters and for myocardial tissue characterization2. The aim of this study was to prospectively assess the predictive value of Cardiac Magnetic Resonance (CMR) parameters for cardiovascular complications in sickle cell disease (SCD) patients.
Methods and materials
We considered 102 white SCD patients (34.38±12.67 years, 53 females) consecutively enrolled in the Myocardial Iron Overload in Thalassemia (MIOT) network3 (Table 1). Myocardial iron overload (MIO) was measured by segmental approach with the multislice multiecho T2* technique (GRE T2* multiecho sequences[10 TEs 2.2-21.8 ms] on 3 parallel short axis views)4. (Fig.1)
Atrial dimensions and biventricular function parameters were quantified in a standard way by cine Steady-State Free Processed (SSFP) images5. (Fig.2)
Results
At baseline CMR only two patients had MIO (global heart T2*< 20 ms).
During a mean follow-up was of 63.29±24.41 months,10 cardiac events (9.8%) were registered: 3 pulmonary hypertension, 1 pulmonary embolism, 1 peripheral vascular disease, 1 transient ischemic attack, 2 supraventricular arrhythmias, 1 heart failure, and 1 death after acute chest syndrome.
CMR predictors of cardiovascular events at univariate analysis were left ventricular ejection fraction and right ventricular mass index (Table 2). Both variables remained independent predictors at multivariate analysis (Table 2).
Conclusion
Reduced left ventricular ejection fraction and increased right ventricular mass index showed a significant prognostic value in patients with SCD. Our data seem to suggest that also CMR could be added as screening tool for identifying SCD patients at high risk for pulmonary and systemic vasculopathy and death.
Personal information and conflict of interest
G. Peritore; Palermo/IT - Author at ARNAS Civico Palermo A. Meloni; Pisa/IT - Author at FTGM Pisa L. Pistoia; Pisa/IT - Author at FTGM Pisa P. Giuliano; Palermo/IT - Author at ARNAS Civico Palermo L. Cuccia; Palermo/IT - Author at ARNAS Civico Palermo M. G. Bisconte; Cosenza/IT - Author at Unità Operativa Ematologia - Centro di Microcitemia, Azienda Ospedaliera Cosenza Presidio Ospedaliero “Annunziata”, Cosenza V. Positano; Pisa/IT - Author at FTGM Pisa D. Messana; Palermo/IT - Author at ARNAS Civico Palermo A. Pepe; Pisa/IT -...
References
1Gladwin MT et al. Hematology Am Soc Hematol Educ Program 2017; 2017(1):423–30.
2Hundley WG et al. Circulation. 2010;121:2462–508.
3Meloni A et al. Int J Med Inform 2009;78:503-12.
4Pepe A et al. J Magn Reson Imaging 2006;23:662-8.
5Marsella M et al. Haematologica 2011;96:515–20.