Impact of liver transplantation on autonomic neuropathy in familial amyloidotic polyneuropathy: an evaluation by spectral analysis of heart rate variability

Abstract

Abstract. Suhr OB, Wiklund U, Ando Y, Ando E & Olofsson B-O (Umeå University Hospital, Umeå, Sweden). Impact of liver transplantation on autonomic neuropathy in familial amyloidotic polyneuropathy: an evaluation by spectral analysis of heart rate variability. J Intern Med 1997; 242: 225-9.Objectives. To evaluate the impact of liver transplantation on familial amyloidotic polyneuropathy type I (FAP) patients' autonomic neuropathy.Design. An open study. Setting. A tertiary referral centre. Subjects. Twelve liver-transplanted FAP patients evaluated before and one year or longer after liver transplantation.Interventions. Spectral analysis of heart rate variability. The low-frequency band after tilting (sympathetic), and the high-frequency band in supine position (parasympathetic) were analysed, as were the pulse and blood pressure reaction to tilting. Clinical symptoms related to autonomic disturbances were recorded. Main outcome measures. Spectral band power for sympathetic and parasympathetic activity. Results.No statistically significant improvements in sympathetic or parasympathetic band power after liver transplantation was found (sympathetic band power: 2.7 (2.2-3.2) before, 2.9 (2.2-3.6) after; parasympathetic 2.0 (1.6-2.4) before and 2.0 (1.7-2.3) after. A significant correlation was noted between orthostatic blood pressure reaction and sympathetic activity before transplantation, but not after the operation. A trend was noted for improved orthostatic blood pressure reaction. Symptomatic improvements in bowel function and orthostatic symptoms were reported by several patients. Conclusions. Although improvements in autonomic symptoms are reported after liver transplantation, no significant improvement is noted in sympathetic or parasympathetic spectral band power of heart rate variability. However, the follow-up period of 17 months may be too short. Further evaluation after an additional two and four years is needed.

Ten-year follow-up of peripheral nerve function in patients with familial amyloid polyneuropathy after liver transplantation

“…In the 6 FAP patients in whom we could examine CV R-R , no apparent improvement in CV R-R was seen during the 10-year post-transplant observation period. Using a spectral analysis of beat-to-beat heart rate variability a similar result was reported in Swedish FAP patients [23]. In addition disturbed gastrointestinal movement is a representative manifestation of autonomic disorders in FAP.…”

“…Earlier reports [25,26] concerning the effects of liver transplantation on FAP emphasized that some autonomic symptoms, including orthostatic hypotension, and bowel and bladder disturbances, were relieved shortly after operation. However, autonomic function tests [23,24] obtained from these patients did not correlate with the improved clinical manifestations.…”

Effects of Orthotopic Liver Transplantation on the Corrected QT Interval in Patients with End-Stage Liver Disease

“…The rapidity of improvement in these areas after OLT suggests that the autonomic dysfunction may be caused by the presence of a circulating neurotransmitter rather than a direct neurotoxin. Indeed, autonomic neuropathy in familial amyloidosis, a known neuropathic disorder, does not improve even 18 months after OLT (16). Table 2 summarizes the findings of recent studies on the QTc before and after OLT.…”

Hereditary and acquired amyloid neuropathies

“…Furthermore, a marked reduction of axonal loss has been shown by histometric comparative study in 7 operated patients as compared with 4 control FAP patients who did not undergo LT [1]. Improvement of autonomic disturbances initially reported [11,27] has not been confirmed more recently in patients with a short [60] or long follow-up [1] by non invasive cardiocirculatory autonomic tests. The general condition improved in half the patients, with weight gain [1].…”