Sickle cell disease (SCD) is a common blood disorder, affecting multiple body systems. SCD was one of the first genetic disorders described and effective treatments have been since developed for many of its manifestations. Nevertheless, cardiovascular complications, which are only now being studied in-depth, are today the leading cause of morbidity and mortality in SCD, with a median lifespan of only 45 years. It is known that SCD patients who develop diastolic dysfunction (increased heart muscle stiffness) are at much higher risk of death. However, the mechanisms of diastolic dysfunction are not well known, preventing the development of effective therapies. Recent work from our group at the University of Chicago suggests that diastolic dysfunction may be due to underlying aortic pathology, rather than intrinsic heart muscle abnormalities. In the proposed study, we will specifically investigate the interaction between diastolic function and changes in aortic properties caused by SCD using the unique, novel software for analysis of cardiovascular magnetic resonance images, recently developed by the Cardiovascular Imaging Team at INSERM in Paris. We anticipate that the results of this study will improve the understanding of the mechanisms of diastolic dysfunction in SCD and thus aid in the development of future treatments to help prolong the lifespan of this patient population.
Project duration : 2 years (2014-2015)
- University of Chicago: V Mor-Avi, Amit Patel
- iCV LIB: N kachenoura, E Bollache