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Objective Supraventricular arrhythmias are commonly detected in patients with anti-mitochondrial antibody M2 (AMA-M2)-associated myopathy. However, the prevalence of supraventricular arrhythmias in unselected AMA-M2-positive patients and the impact of AMA-M2 on supraventricular arrhythmias have yet to be fully investigated. Methods We analyzed 384 patients (116 men; age, 60 [48-69] years), who underwent AMA-M2 testing following the detection of elevated hepatobiliary enzymes. Supraventricular arrhythmias involving atrial fibrillation, atrial flutter, atrial tachycardia, sick sinus syndrome, and atrial standstill were confirmed by a 12-lead electrocardiogram, 24-hour ambulatory monitoring, and physician-assigned diagnoses within the three years before and two years after the AMA-M2 test. Results Seventy-seven (20%) patients were positive for AMA-M2. The prevalence of supraventricular arrhythmias among AMA-M2-positive patients was higher than that among AMA-M2-negative patients (14% vs. 6%, p=0.008). A univariate analysis showed that supraventricular arrhythmias were associated with AMA-M2 positivity, aging, congestive heart failure, and the CHADS 2 score. The multivariate analysis determined that AMA-M2 positivity was an independent risk factor for supraventricular arrhythmias (odds ratio 3.52, p=0.011). Among the AMA-M2-positive patients, the AMA-M2 titer did not differ to a statistically significant extent, regardless of the presence or absence of supraventricular arrhythmias. Multiple supraventricular arrhythmias with extremely low atrial deflections was a characteristic finding in AMA-M2-positive patients with supraventricular arrhythmias. Conclusion AMA-M2 enhances the risk of supraventricular arrhythmias, indicating the possible involvement of the atrial myocardium and the formation of an arrhythmogenic substrate. The results highlight the need for clinical attention to supraventricular arrhythmias in AMA-M2-positive patients.

Background: The precise location of truly active reentry circuits of typical atrial flutter (AFL) has not been well identified. The purpose of this study was to verify our hypothesis that the posterior block line is located along the posteromedial right atrium (PMRA) and the crista terminalis (CT) is the anterior pathway of AFL, with real-time intracardiac echo (ICE)., Methods: The entire right atrium (RA) three-dimensional activation and entrainment mapping were evaluated during AFL in 18 patients using CARTO sound., Results: The CT was clearly visualized by ICE and the local electrograms along the CT were single potentials in all the patients. The CT was recognized as the truly active anterior pathway based on entrainment mapping in all patients. Double potentials were recorded along the PMRA. Entire RA entrainment mapping could be performed in 16 patients. The reentry circuits were separated into three passages. The first was around the tricuspid annulus (TA), the second the anterior superior vena cava (SVC; AFL waves passed between the anterior SVC and RA appendage), and the last the posterior SVC (between the posterior SVC and upper limit of the PMRA). All three of these passages were active in four, around the TA and anterior SVC in eight, around the TA and posterior SVC in three, and around only the anterior SVC in one patient., Conclusions: The CT functions as the anterior pathway of typical AFL, and the posterior block line was located along the PMRA. Dual or triple circuits were recognized in the majority of AFL patients., (© 2015 Wiley Periodicals, Inc.)

Introduction: Conduction block in the posterior right atrium (RA) plays an important role in perpetuating atrial flutter (AFL). Although conduction blocks have functional properties, it is not clear how the block line changes with the pacing rate, pacing site, and administration of antiarrhythmic drugs., Methods and Results: Forty patients with typical AFL were enrolled. Pacing (110, 170, 230 ppm) from the coronary sinus ostium (CSo) and low lateral RA was performed. After 1 mg/kg pilsicainide (pure sodium channel blockade) administration, the pacing protocol was repeated. Conduction block was assessed based on a color-coded isopotential map and 20 points of virtual unipolar electrograms in the posterior RA using noncontact mapping. Block line proportion was defined as the percentage of length of the block line between the superior and inferior vena cava. The pacing rate-dependent extension of the block proportion was significant during pacing from both sides (pacing from the CSo: 59 ± 17% at 110 ppm, 69 ± 16% at 230 ppm, P < 0.05; pacing from the low lateral RA: 43 ± 19% at 110 ppm, 55 ± 22% at 230 ppm, P < 0.05). The block line was significantly longer during CSo pacing than during low lateral RA pacing at each rate (all P < 0.05). After pilsicainide administration, the block line extended further., Conclusion: In addition to pacing rate-dependent and site-dependent changes in the block line, pilsicainide further extended the block line length. This phenomenon explains the clinical observation that counterclockwise AFL occurs more frequently than clockwise AFL, and the mechanism of class IC AFL., (© 2012 Wiley Periodicals, Inc.)

Background: Noncontact mapping is useful for the diagnosis of various arrhythmias. Virtual unipolar electrogram morphologies (VUEM) of the conduction block and the turnaround points, however, are not well defined. We compared the VUEM characteristics of a transverse conduction block in the posterior right atrium (RA) with those of contact bipolar electrograms obtained during typical atrial flutter (AFL)., Methods: Contact bipolar electrograms were used to map the posterior RA during typical AFL in 16 patients. Twenty points of the VUEM recorded along the block line were analyzed and compared with contact bipolar electrograms., Results: Seventeen AFLs were analyzed. Fifteen AFLs showed an incomplete transverse conduction block in the posterior RA by contact bipolar mapping. A double potential on the block line corresponded to the two components of the VUEM, in which the second component showed an Rs, RS, or rS pattern. At the turnaround point, a fused double potential of the contact bipolar electrograms corresponded to a change of the second component of the VUEM from an rS to a QS morphology. Two AFLs showed a complete block line in the posterior RA. The contact bipolar electrogram showed double potentials from the inferior vena cava to the superior vena cava, whereas the second component of the VUEM remained in an unchanged Rs, RS, or rS pattern., Conclusion: VUEM analysis was a reliable method for identifying the posterior block line during AFL. This method may also be applicable for detecting block lines and turnaround points of circuits in other unmappable arrhythmias.

We describe a case of a 60-year-old male with dilated phase of hypertrophic cardiomyopathy caused by Fabry disease. He was diagnosed to have a cardiac variant of Fabry disease by an enzyme assay and a right ventricular endomyocardial biopsy which revealed specific features of this disease and cardiac involvement was the sole manifestation. He has developed dilated cardiomyopathy with sustained atrial flutter and frequent non-sustained ventricular tachycardia requiring isthmus ablation and cardiac resynchronization therapy with defibrillator.

Background: The posteromedial right atrium (PMRA) forms a block line during typical atrial flutter (AFL). However, whether upper turnover portion exists at the anterior or posterior superior vena cava (SVC) has not been determined., Methods: We performed right atrial mapping during AFL in 20 patients (typical AFL, n = 17; reverse typical AFL, n = 3) using an electroanatomical mapping system., Results: Mean AFL cycle length was 224 +/- 20 ms and mean number of mapping points was 140 +/- 27. PMRA formed a block line during both typical and reverse AFL in all patients. However, in 16 of 17 patients mapped with typical AFL, PMRA did not extend superiorly to the orifice of the SVC and AFL wave propagated between the upper limit of the PMRA and the posterior SVC. In the remaining patient mapped with typical AFL, a double potential was recorded along the PMRA continuously between the orifices of the inferior vena cava (IVC) and SVC. In the three patients mapped with reverse typical AFL, a posterior barrier was detected from IVC to the upper limit of the PMRA and AFL wave propagated between the upper limit of the PMRA and the posterior SVC. Mean length from IVC to upper limit of the PMRA was 81 +/- 8% of the length from IVC to SVC., Conclusions: PMRA forms a functional block line during both typical and reverse typical AFL. The upper turnover portion of reentry circuit for AFL was observed between the upper limit of the PMRA and the posterior SVC in the majority of isthmus-dependent AFL patients.

The crista terminalis (CT) is reportedly a critical barrier for maintaining typical atrial flutter (AFL), but recent observations have suggested the presence of posteromedial functional block, as well as crista conduction. Therefore, this study was designed to identify the correlation between the posterior boundary of AFL and anatomical architecture in the human right atrium (RA) using 3-dimensional (D) intracardiac echocardiography (ICE). In 11 patients with AFL (typical 9, reverse typical 2), mapping with a 10-pole (n=5) or 32-pole (n=6) catheter was performed during AFL. ICE was used to determine the catheter's position relative to the intra-atrial structures. In all patients, double potentials were recorded at the posteromedial RA and the catheter positions were recognized as posterior to the CT by 3-D ICE. Double potentials were not recorded on the CT, and the activation sequence revealed a craniocaudal direction in the 9 patients with typical AFL and caudocranial direction in the 2 patients with reverse typical AFL. These findings demonstrate that the posterior boundary of the AFL circuit is in the sinus venosa region posterior to the CT, which may provide an important insight into the mechanism of maintaining AFL.