Late ventricular potentials in familial Mediterranean fever with and without AA amyloidosis

Abstract

Data on increased rhythm and conduction disturbances in FMF are limited and conflicting. For instance, Akcay et al. (13) found that QT dispersion (QTd) and corrected dispersion (QTcd), which reflect cardiac repolarization heterogeneity and thereby suggest predisposition for cardiac arrhythmias, are increased in uncomplicated FMF. However, different methodologies have yielded normal QTd results in both uncomplicated and amyloidosis-affected FMF patients (14, 15). Limited information is found on depolarization-associated markers for arrhythmias. Low-amplitude, high-frequency waves-termed late ventricular potentials (LPs)-can be detected immediately adjacent to the QRS complex by signal-averaged electrocardiography Udi Nussinovitch 1 , Avi Livneh 2,3Original Article Abstract 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.

The Impact of Osteocalcin, Osteoprotegerin and Osteopontin on Arterial Stiffness in Chronic Renal Failure Patients on Hemodialysis

“…Furthermore, OP plasma levels were positively correlated with VS in patients with coronary disease but not in young subjects without clinical symptoms or risk factors of atherosclerosis [43, 44]. Interestingly, OP levels could be reduced by statins and angiotensin II blockade [45]. …”

Evaluation of Cardiac Arrhythmia Susceptibility in Pediatric Familial Mediterranean Fever Patients

“…While FMFrelated pericarditis and cardiac amyloidosis are the most commonly expected complications, recent studies have reported that FMF patients can suffer sudden cardiac death or malign arrhythmias that are caused by cardiac repolarization abnormalities, even without the presence of amyloidosis. [4,5] This has been linked to the chronic inflammation occurring in FMF. It is believed that the chronic inflammation observed in FMF can lead to endothelial injury and ischemic cardiovascular damage.…”

“…[12,13] There are many studies in the literature which have used these new indices that indicate ventricular repolarization in FMF. [4,5,[14][15][16] Studies have revealed that the Tp-e interval is superior to QT dispersion in foreseeing sudden cardiac death and ventricular arrhythmia. [17] Our aim in this study was to evaluate the longstanding as well as the newly developed electrocardiogram parameters indicating myocardial repolarization and to assess the QT interval, corrected QT interval, Tp-e interval, corrected Tp-e, Tp-e/corrected QT, P wave dispersion to see whether pediatric patients diagnosed with FMF have a predisposition toward arrhythmia.…”

Relation Between Familial Mediterranean Fever and QT Markers (QTc, QTd, and QTcd): A Systematic Review and Meta-Analysis

“…For instance, its prevalence in Turkey is estimated to be one in 1,000 children. Mediterranean fever (MEFV) gene [1][2][3] mutation is thought to be responsible for its occurrence.…”