Blood Pressure Variability in Fabry Disease Patients

Nephron. 2022;146(4):343-350. doi: 10.1159/000520699.

PMID: 34933310

Mevlut Tamer Dincer, Seyda Gul Ozcan, Baris Ikitimur

Highlights: In this study, it is aimed to examine the circadian variations of blood pressure (BP) and heart rate variability in Fabry disease patients.

Abstract

Introduction and Objective: Fabry disease is a rare multisystemic, lysosomal storage disorder with X-linked inheritance. The autonomic nervous system's participation is well understood, but information on how the blood pressure (BP) and heart rate vary in Fabry disease is mostly lacking. This study describes the circadian fluctuations in blood pressure and heart rate variability in Fabry disease patients.

Methods: Between July 2019 and March 2020, 31 adult (>18 years) Fabry disease patients were enrolled who were routinely followed up in the outpatient clinic: 16 males and 15 females. Echocardiography and ambulatory blood pressure monitoring are conducted on each subject. As measures of variability, it was employed the standard deviation (SD), coefficient of variation (CV), and average real variability. Two control groups are created utilizing age, sex, and eGFR data in the first group and antihypertensive medication use in the second group for propensity score matching.

Results: Except for the nighttime systolic BP, all BP measures in the FD group were significantly lower than those in the control groups. FD patients and controls had comparable non-dipping and reverse dipping statuses. None of the BP variability measurements were higher in FD patients, according to the findings of this study. When it comes to heart rate variability statistics, FD patients' nighttime SD and CV were significantly lower than those of the controls'.

Conclusion: An early indicator of autonomic involvement in FD may be a decrease in heart rate variability rather than an increase in blood pressure fluctuation.

Keywords: Ambulatory blood pressure monitoring, Blood pressure, Fabry disease