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Late ventricular potentials in familial Mediterranean fever with and without AA amyloidosis
Udi Nussinovitch1, Avi Livneh2,3
1Medicine A, Rambam Health Care Campus, Haalia Hashnia, Haifa, Israel.
Objective:
Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by episodic and chronic inflammation that may lead to both accelerated coronary atherosclerosis and cardiac AA amyloidosis. We hypothesized that late ventricular potentials (LPs), an established electrocardiographic susceptibility marker of ventricular arrhythmias, will be more common in FMF than in the adjusted normal population due to these two types of inflammation-associated cardiac effects. Therefore, we aimed to evaluate the occurrence of LPs in FMF patients with and without amyloidosis.
Material And Methods:
Signal-averaged electrocardiography was performed in consecutive patients with FMF using the Frank corrected orthogonal lead system. At least 200 consecutive beats were digitally recorded and averaged, and the presence of LPs was determined according to acceptable thresholds.
Results:
There were 54 patients with colchicine-treated FMF, of whom 14 had biopsy-proven AA amyloidosis. None of the uncomplicated FMF patients and 2 of the 14 FMF amyloidosis patients had abnormal or borderline LPs.
Conclusion:
Based on LPs as a susceptibility marker for arrhythmia, FMF patients, including the large majority of FMF patients with amyloidosis, are seemingly not at an increased risk to develop arrhythmias.
Insights
Familial Mediterranean fever (FMF) patients, even those with amyloidosis, do not show an increased risk for arrhythmias. Late ventricular potentials (LPs), a marker for arrhythmia susceptibility, were rarely found in this FMF cohort.
Area of Science:
- Cardiology
- Genetics
- Rheumatology
Background:
- Familial Mediterranean fever (FMF) is an autosomal recessive inflammatory disease.
- FMF can lead to accelerated coronary atherosclerosis and cardiac AA amyloidosis.
- Late ventricular potentials (LPs) are electrocardiographic markers for ventricular arrhythmia susceptibility.
Purpose of the Study:
- To evaluate the occurrence of LPs in FMF patients.
- To compare LP prevalence in FMF patients with and without AA amyloidosis.
- To assess arrhythmia risk in FMF based on LP presence.
Main Methods:
- Signal-averaged electrocardiography (SAECG) was performed on 54 colchicine-treated FMF patients.
- The Frank corrected orthogonal lead system was used for recordings.
- LPs were identified using established thresholds in averaged electrocardiographic signals.
Main Results:
- 14 out of 54 FMF patients had biopsy-proven AA amyloidosis.
- No uncomplicated FMF patients exhibited abnormal or borderline LPs.
- Only 2 of the 14 FMF patients with amyloidosis had abnormal or borderline LPs.
Conclusions:
- FMF patients, including those with AA amyloidosis, do not appear to have an increased risk of arrhythmias.
- LPs were found to be uncommon in this cohort of FMF patients.
- The study suggests that FMF itself, or its associated amyloidosis, may not significantly elevate arrhythmia risk as indicated by LPs.
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