Abstract TP191: Three-month Daily Blood Pressure Monitoring Predicts Incident Stroke In CADASIL

Abstract

Introduction: CADASIL is a hereditary small vessel disease caused by NOTCH3 mutations. Whether superimposed hypertension had harmful effects on these populations was unknown. Methods: We recruited 100 participants in the prospective cohort of Taiwan CADASIL Registry with documented NOTCH3 mutations , as well as 18 age- and sex-matched controls. Daily blood pressure (BP) and heart rate (HR) were recorded by the participants with a standardized home-based sphygmomanometer for consecutive 90 days. Linear analysis of the variability parameters was done. Neuroimaging markers of small vessel disease on brain magnetic resonance imaging and any incident stroke was documented. Results: Participants with NOTCH3 mutations are more likely to have stroke history and use antithrombotic or antihypertensive medications than controls. However, the systolic, diastolic BP, and pulse pressure were comparable, except for higher mean HR (77.3±10.0 vs 70.7±8.3, P =0.01) in the NOTCH3 group. In participants with NOTCH3 mutations, higher 90-day mean systolic and diastolic BP correlated with more cerebral microbleeds, while the mean, standard deviation, coefficient of variation, and average real variability of 90-day HR positively correlated with number of lacunes. During median follow-up of 24 months, there were 8 stroke events (5 ischemic, 3 hemorrhagic). Participants with persistent hypertension (90-day mean systolic/diastolic BP > 130/80 mmHg) were more likely to have incident stroke (log-rank P = 0.04), and the effects persisted (hazard ratio 5.29, 95% CI 1.12 - 25.1) after adjustment of number of cerebral microbleeds. Conclusions: In participants with NOTCH3 mutations, persistent hypertension had detrimental effects on the severity of cerebral microbleeds as well as incident stroke. Aggressive blood pressure control seems beneficial in hereditary small vessel disease.