Proposed diagnostic criteria for arrhythmogenic cardiomyopathy: European Task Force consensus report.

Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disease characterized by prominent "non-ischemic" myocardial scarring predisposing to ventricular electrical instability. Diagnostic criteria for the original phenotype, arrhythmogenic right ventricular cardiomyopathy (ARVC), were first proposed in 1994 and revised in 2010 by an international Task Force (TF). A 2019 International Expert report appraised these previous criteria, finding good accuracy for diagnosis of ARVC but a lack of sensitivity for identification of the expanding phenotypic disease spectrum, which includes left-sided variants, i.e., biventricular (ABVC) and arrhythmogenic left ventricular cardiomyopathy (ALVC). The ARVC phenotype together with these left-sided variants are now more appropriately named ACM. The lack of diagnostic criteria for the left ventricular (LV) phenotype has resulted in clinical under-recognition of ACM patients over the 4 decades since the disease discovery. In 2020, the "Padua criteria" were proposed for both right- and left-sided ACM phenotypes. The presently proposed criteria represent a refinement of the 2020 Padua criteria and have been developed by an expert European TF to improve the diagnosis of ACM with upgraded and internationally recognized criteria. The growing recognition of the diagnostic role of CMR has led to the incorporation of myocardial tissue characterization findings for detection of myocardial scar using the late­gadolinium enhancement (LGE) technique to more fully characterize right, biventricular and left disease variants, whether genetic or acquired (phenocopies), and to exclude other "non-scarring" myocardial disease. The "ring-like' pattern of myocardial LGE/scar is now a recognized diagnostic hallmark of ALVC. Additional diagnostic criteria regarding LV depolarization and repolarization ECG abnormalities and ventricular arrhythmias of LV origin are also provided. These proposed upgrading of diagnostic criteria represents a working framework to improve management of ACM patients.

The predictive role of early recurrences of atrial arrhythmias after pulmonary vein cryoballoon ablation. Is blanking period an outdated concept? Insights from 12-month continuous cardiac monitoring.

BACKGROUND: Early recurrences of atrial arrhythmias (ERAA) after atrial fibrillation (AF) catheter ablation do not predict procedural failure. A well-demarcated homogeneous lesion delivered by cryoballoon is less arrhythmogenic, and the recommended three-months blanking period may not refer to cryoballoon ablation (CBA). OBJECTIVE: We aimed to evaluate the predictive role of ERAA after second-generation CBA using an implantable loop recorder. METHODS: This prospective observational study enrolled 100 patients (58 males, median age 58) with paroxysmal/persistent AF undergoing pulmonary vein (PV) CBA using second-generation cryoballoon with simultaneous ECG loop recorder implantation. The duration of follow-up was 12 months, with scheduled visits at 3, 6 and 12 months. RESULTS: 99 patients from 100 completed the 12-month follow-up period. ERAA occurred in 31.3 % of patients. 83.9 % of patients with ERAA also developed late recurrences. The 12-month freedom from AF in patients with ERAA was significantly lower than in those without ERAA (p < 0.0001). Non-paroxysmal AF and longer arrhythmia history were associated with increased risk of both early (HR 3.27; 95 % CI 1.32-8.08; p = 0.010 and HR 1.0147; 95 % CI 1.008-1.086; p = 0.015, respectively) and late recurrences (HR 3.89; 95 % CI 1.67-9.04; p = 0.002 and HR 1.0142; 95 % CI 1.007-1.078; p = 0.019, respectively) of AF. ERAA were another predictor for procedural failure (HR 15.2; 95 % CI (6.42-35.99; p = 0.019). CONCLUSIONS: ERAA occurred in the third of the patients after PV second-generation CBA and are strongly associated with procedural failure. Longer duration of AF history and persistent AF are independent predictors of AF's early and late recurrence.

Radiofrequency versus Cryoballoon Ablation of Atrial Fibrillation: An Evaluation Using ECG, Holter Monitoring, and Implantable Loop Recorders to Monitor Absolute and Clinical Effectiveness.

INTRODUCTION: While several studies have compared the radiofrequency current (RFC) and cryoablation for the treatment of patients with atrial fibrillation (AF), no study has monitored the long-term outcomes with the usage of implantable loop recorders (ILRs). METHODS: We enrolled 89 consecutive patients with nonvalvular paroxysmal AF (N = 44 for RFC and N = 45 for cryoballoon). The primary efficacy end point was the assessment of effectiveness for each group (RFC versus cryoballoon) when examining freedom from arrhythmia by monitoring with ECG, Holter, and implantable loop recoder (ILR). The primary safety end point compared rates of adverse events between both groups. The secondary efficacy end point examined the duration of the postablation blanking period from ILR retrieved data. RESULTS: The mean age of the study population was 56.6 ± 10.2 years, and the follow-up duration was 12 months. There were no differences in baseline patient characteristics between groups. At 12 months, the absolute effectiveness (measured by ILR) was 65.9% in the RFC group and 51.1% in the cryoballoon group (OR = 1.85; 95% CI: 0.79-4.35; p = 0.157), and the clinical effectiveness (measured by ECG and Holter) was 81.8% in the RFC group and 55.6% in the cryoballoon group (OR = 3.6; 95% CI: 1.37-9.46; p = 0.008). There was no difference in safety between both groups. Asymptomatic episodes were significantly more present in the RFC group as measured by ILRs (p < 0.010). In cryoballoon group, arrhythmia episodes were recorded equally irrespective of the follow-up method (i.e., ECG and Holter versus ILR (p > 0.010)). The blanking period does not seem to be as important in cryoballoon as compared to RFC. CONCLUSION: RFC and cryoballoon ablation had similar absolute effectiveness at 12 months. ECG and Holter were effective when assessing the efficacy of the cryoballoon ablation; however, in the RFC group, ILR was necessary to accurately assess long-term efficacy.

Sodium-channel blockers might contribute to the prevention of ventricular tachycardia in patients with long QT syndrome type 2: a description of 4 cases.

Four patients with long QT type 2, aged 11 to 18 years from unrelated families, with recurrent syncope and polymorhic ventricular tachycardia were studied. Long QT syndrome was diagnosed in these children at ages 4 to 7 years. Syncope, QT prolongation on electrocardiogram (corrected QT interval ≥ 490 milliseconds), notched T-wave morphology, bradycardia, and polymorphic ventricular arrhythmia were found in all of the patients. The KCNH2-L586M; KCNH2-G604S, KCNH2-L1045F; and a combined mutation KCNH2 T613M + SCN5A R190G were genotyped. Syncope, implantable cardioverter-defibrillator shocks, and tachycardia persisted in these patients, although they were receiving a full dose of ß-blocker therapy. Adding a sodium-channel blocker (IC class) led to a reduction in the polymorphic ventricular arrhythmia. No syncope episodes were registered during the patients' 8 to 60 months of follow-up on the combined antiarrhythmic therapy. Further studies are needed to better define the possible role of sodium-channel blockers in patients with long QT type 2.

Atrial appendage transcriptional profile in patients with atrial fibrillation with structural heart diseases.

During the last few years DNA microarray studies of gene expression changes in human atrial tissues from patients with and without atrial fibrillation (AF) have been performed. For this purpose, tissue samples are usually collected from AF patients undergoing open heart surgery. These investigations have limitations associated with the unavoidable heterogeneity of compared groups which is due to the presence of various structural changes accompanying different sets of underlying heart diseases in both groups. It is thus reasonable to compare the atrial tissue samples from AF patients with those from individuals without signs of cardiovascular disease. To address this, we selected the atrial tissue samples from 12 AF patients (who underwent open heart surgery) and compared them with control atrial tissue samples from 10 individuals with no signs of cardiovascular diseases (those who died due to street accident). cDNA microarray method and reverse transcription-polymerase chain reaction (RT-PCR) analysis were used to identify genes which can discriminate between control and pathologically altered atrial tissues. Thirty-nine genes were found to be differentially expressed in pathologically altered tissues samples independently of the type of the underlying structural heart disease. These genes are involved in signal transduction, gene transcription regulation, cell proliferation, and apoptosis. The greatest alterations were observed for NOR1, DEC1, MSF, and Bcl2A1 genes (5 to 28-fold decrease, P < 0.05). Additional studies are needed to determine the specific role of each selected gene in pathophysiological changes leading to AF.