Electrocardiographic Findings in Familial Amyloidotic Polyneuropathy

Abstract

The electrocardiographic findings in patients with verified familial amyloidotic polyneuropathy, from a defined population in northern Sweden with an extremely high prevalence of this disease, were studied. The first electrocardiogram registered, after the diagnosis has been established, was reviewed in 105 patients. In 61 patients, there was at least 1 additional electrocardiogram registered more than 3 years after the first, and the latest of these were also reviewed. Mean follow-up time was 8.2 years. The proportion of patients with an abnormal or suspected abnormal electrocardiogram was 54% at the initial examination and 80% at follow-up. The most common findings at onset of symptoms were atrioventricular and intraventricular conduction abnormalities (33%), S-T depression (20%), flattened or inverted T waves (22%) and prolongation of the Q-T interval (17%). The prevalence of conduction and S-T-T abnormalities increased further during the follow-up period. The occurrence of all electrocardiographic abnormalities was age-related, and there were few abnormalities in patients younger than 55 years. The cardiac conduction disturbances were progressive, and 19

Liver Transplantation Does Not Prevent the Development of Life-Threatening Arrhythmia in Familial Amyloidotic Polyneuropathy, Portuguese-Type (ATTR Val30Met) Patients

“…The only published longitudinal study of ECG changes in FAP patients showed that during a mean follow-up time of 8 years, the prevalence and severity of the conduction disturbances markedly increased and that 19 of 61 (31%) patients required pacemaker treatment during the observation period (18). This is comparable with 4 of 17 (24%) patients in this study over a 5-year period.…”

“…There are also recent reports of a rapidly progressing cardiomyopathy after liver transplantation for certain TTR mutations (15)(16)(17). An increased prevalence and severity of cardiac arrhythmias and conduction disturbances are the main heart complications in FAP ATTR Val30Met, and the natural course of the disease often leads to the insertion of a pacemaker (18). Cardiac failure is very seldom observed in FAP ATTR Val30Met patients, and none of our patients receiving transplants have developed symptoms consistent with heart failure (19).…”

New insights into the clinical evaluation of hereditary transthyretin amyloidosis patients: a single center's experience

“…107,108 Different cross-sectional studies, mainly based on conventional electrocardiograms (ECGs), have revealed a high prevalence of conduction disturbances, intermittent atrial and ventricular arrhythmias, and signs of low voltages in the ECGs. [109][110][111][112] Development of severe arrhythmia is common in V30M patients. A longitudinal study of 12-lead ECG changes in non-transplant ATTR patients over a mean follow-up period of 8 years showed that 19 (31%) of 61 patients required pacemaker implantation during the observation period.…”

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

“…Pacemaker treatment is frequently used, and with wider indication, as autonomic dysfunction increases the risk of life threatening Adams-Stokes attacks. 27,28 Patients with frequent non-sinus beats also exhibited BP overshoot after tilt reversal. Although this finding may be related to their arrhythmia, we cannot exclude that these patients also had an abnormal cardiac autonomic regulation, but the latter could not be detected as their heart arrhythmia prevented HRV analysis.…”