Blood pressure overshoot after tilt reversal in patients with familial amyloidotic polyneuropathy

• The pathophysiology of the hemodynamic responses to postural stress in familial amyloidotic polyneuropathy (FAP) remains to be elucidated. The aim of the study was to evaluate hemodynamic responses after tilt reversal in FAP. Systolic blood pressure (BP) and heart rate variability (HRV) were analyzed in the baseline, 70° upright position, and after tilt reversal in 15 FAP patients and 14 healthy controls. Beat-to-beat BP was recorded with a Finapres device. Maximum systolic BP after tilt reversal was increased with 22±13 mm Hg in FAP patients as compared with baseline (BP overshoot), whereas controls showed a significantly lower BP overshoot (8±6 mm Hg, P<0.001). In all states, total spectral power and the power of the low and high frequency components were all significantly lower than those of the controls (P<0.01). In a linear regression analysis adjusted for age, we found a significant inverse relation between BP overshoot and HRV (total spectral power, power of the low-frequency and high-frequency components) in all three states (standardized β between -0.74 to -0.53, P<0.01). Five FAP patients presented a trial arrhythmia precluding HRV analysis: four of those presented BP overshoots 12 mm Hg. BP overshoot may be a marker to assess the progression of cardiac autonomic dysfunction, especially as heart arrhythmia in many FAP patients prevent HRV analysis. In addition, assessment of the post-tilt BP reaction points to possible treatment modalities for orthostatic hypotension at least in the early stages of the disease.

Nyckelord

autonomic nervous system

blood pressure

familial amyloidotic polyneuropathy (FAP)

heart rate variability (HRV)

transthyretin (TTR)

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