Cerebrovascular and cardiovascular autonomic regulation in sickle cell patients with white matter lesions

Background The prevalence of asymptomatic white matter lesions (WML) in patients with sickle cell disease (SCD) has been described to be very frequent in young adults. Cerebrovascular regulation and cardiovascular autonomic regulation, more specifically the sympatho-vagal balance can be altered in SCD. In this study we assessed the association between WML, cerebrovascular regulation and sympatho-vagal balance in SCD. Method Adults with no history of stroke from a cohort of SCD patients were prospectively evaluated for, cerebrovascular regulation using Mx for autoregulation, breath holding test for cerebrovascular reactivity and cerebral arterial compliance calculated from arterial blood pressure and cerebral velocities. Sympatho-vagal balance was assessed using heart rate variability parameters. WML was assessed with MRI using Fazekas score grading and the presence of lacunar lesions. Results Forty-one patients (F/M:25/16) were included. Median age was 37.5 (range 19-65). Twenty-nine (70,7%) patients had SS genotype, 7 patients (17,1%) had SC genotype and 5 (12,2%) patients had Sß° genotype. Among the 41 patients included, 11 patients had WML (26,8%). Patients with WML were significantly older (44.5 vs 30.6 years; p<0.001), had a lower HF (HF 157 ms 2 vs HF 467.6 ms 2 ; p<0.005) and impaired cerebral arterial compliance (CaBVR 15.4 vs 37.3 cm 3 /mmHg; p<0.014). Cerebral blood flow velocities, reactivity to breath holding test and cerebral autoregulation parameters did not significantly differ between the two groups. Conclusions Lower parasympathetic activity and impaired cerebral arterial compliance were associated with WML in adults with SCD. This could potentially yield to a better understanding of pathophysiological parameters leading to premature cerebrovascular ageing in SCD patients.