title: Continuous development of arrhythmia is observed in Swedish transplant patients with familial amyloidotic polyneuropathy (amyloidogenic transthyretin Val30Met variant).
abstract: In patients with familial amyloidotic polyneuropathy (FAP), heart complications are prognostic factors for mortality and morbidity after liver transplantation (LT). However, only a few studies have analyzed the development of arrhythmia in transplant patients with FAP. We investigated the development of arrhythmia requiring pacemaker insertion (PMI) in Swedish transplant patients with FAP, and we related the findings to gender, age at disease onset, and survival. One hundred four transplant patients with the amyloidogenic transthyretin Val30Met mutation were included in the study. Twenty-six (25%) received a pacemaker during the observation period (a median of 11 years after disease onset). This frequency was comparable to that noted in a previous study describing the natural course of FAP. No significant differences in PMI between early-onset cases (<50 years old) and late-onset cases (≥ 50 years old) or between genders were observed. PMI was not significantly related to patient survival. Our study confirms our previously reported short-time observation: LT does not prevent the development of heart arrhythmia necessitating PMI. The development of arrhythmia is unrelated to gender or age at disease onset, and the yearly risk does not appear to decrease with time after LT.
authors.affiliation: Departments of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden.
authors.forename: Sadahisa
authors.initials: S
authors.lastname: Okamoto
authors.forename: Rolf
authors.initials: R
authors.lastname: Hörnsten
authors.forename: Konen
authors.initials: K
authors.lastname: Obayashi
authors.forename: Priyantha
authors.initials: P
authors.lastname: Wijayatunga
authors.forename: Ole B
authors.initials: OB
authors.lastname: Suhr
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D001145:Arrhythmias, Cardiac / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D002304:Cardiac Pacing, Artificial; D016009:Chi-Square Distribution; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011228:Prealbumin / Q000235:genetics*; D016016:Proportional Hazards Models; D018570:Risk Assessment; D012307:Risk Factors; D013548:Sweden / Q000453:epidemiology; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D011228:Prealbumin