HYPERTENSION, AUTONOMIC DYSFUNCTION AND SUDDEN DEATH RISK IN CADASIL DISEASE: NOVEL DATA

Abstract

Objective: Sudden death (SD) is the first cause of mortality in CADASIL disease (CD)(Cerebral Autosomic Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy). An early development of cardiac autonomic neuropathy (CAN) in the pathogenesis of CD was demonstrated by others. As we detected hypertension in members of 4 families of this rare condition we decided to explore the imbalance of the autonomic nervous system as a potential tool for the prediction of SD risk. Aims: to explore the relationship between hypertension and CAN in CD. Design and method: We included 10 hypertensive (officce and 24 hs ABPM) patients with CD. Autonomic function was evaluated by the study of heart rate variability (HRV) and Ewing test (handgrip, inspiration/espiration, valsalva manouver and orthostatism). CAN stages were determined as as subclinical, early and advanced. Results: CD patients were classified as hypertensives (n:5), or normotensives. Table 1 showed hypertensives subjects characteristics and the distribution of CAN. The control group was constituted with patients with CD and arterial normotension (n:5), the study of HRV and Ewing Test being normal in them. No association was observed between the number and extent of brain lessions, location, impact of white matter with the presence of hypertension. The prevalence of CVRF was similar between both groups. Conclusions: Autonomous alterationwas identified in 80% of hypertensive patients with CADASIL disease in our population sample. This association would be independent of traditional CVRF, and the evolutionary characteristics of brain lesions. Although hypertension has not been reported elsewhere as associated with CD, its identification could indicate the risk of autonomic nervous system involvement in these patients. The relevance of their suspicion would reside in the risk re-stratification of cardiovascular events with a focus on the risk of sudden death, linked to the NAC. The limitation of the reduced number of subjects requires the analysis of this hypothesis in a larger population sample, the present being a proof of concept that could justify the development of a survey work in other centers that report cases of this rare disease.