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Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
QT variability in amyloidosis of familial Mediterranean fever
Udi Nussinovitch1, Ilan Ben-Zvi, Avi Livneh
1Israel Naval Medical Institute, Israel Defense Forces Medical Corps, Haifa, Israel.
Background:
The association between familial Mediterranean fever (FMF) and increased risk for ventricular arrhythmias is controversial, and data on this subject are meager.
Objectives:
To evaluate QT variability index (QTVI) and other repolarization markers associated with arrhythmogenity in patients with amyloidosis of FMF.
Methods:
The study group comprised 12 FMF patients with amyloidosis, and 14 age and gender-matched healthy subjects served as the control group. QT measurements were conducted according to accepted procedure, using computerized software for recording and analysis.
Results:
No differences were found in clinical and demographic parameters in the study and control groups, except for hypertension which was more common in the FMF amyloidosis group. QTc and power spectral analysis of QT variability parameters were similar in both groups. Nevertheless, QTVI values in FMF amyloidosis patients were significantly higher than in healthy individuals (-1.02 +/- 0.38, vs. -1.36 +/- 0.32 respectively, P = 0.02).
Conclusions:
Compared with healthy controls, amyloidosis of FMF is associated with increased QTVI. It remains unknown whether this finding is solely amyloidosis related and whether it has any prognostic significance.
Insights
Familial Mediterranean Fever (FMF) with amyloidosis shows higher QT variability index (QTVI), a marker of heart rhythm issues. This finding may indicate increased arrhythmia risk in these patients.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- The link between FMF and ventricular arrhythmias is not well-established.
- Amyloidosis is a potential complication of FMF, affecting organs including the heart.
Purpose of the Study:
- To assess QT variability index (QTVI) and other repolarization markers in FMF patients with amyloidosis.
- To investigate potential arrhythmogenic markers in FMF-associated amyloidosis.
- To compare repolarization markers between FMF amyloidosis patients and healthy controls.
Main Methods:
- The study included 12 FMF patients with amyloidosis and 14 healthy controls.
- QT measurements were performed using computerized software.
- QT variability index (QTVI) and other repolarization parameters were analyzed.
Main Results:
- No significant differences in QTc or power spectral analysis of QT variability were observed between groups.
- QTVI was significantly higher in FMF amyloidosis patients compared to healthy controls (P=0.02).
- Hypertension was more prevalent in the FMF amyloidosis group.
Conclusions:
- Amyloidosis associated with FMF is linked to an elevated QTVI.
- The clinical significance and prognostic value of increased QTVI in this population require further investigation.
- The findings suggest a potential association between FMF amyloidosis and cardiac repolarization abnormalities.
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