Brugada Syndrome and Exercise Practice: Current Knowledge, Shortcomings and Open Questions

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Análisis del ECG para la detección automática del patrón característico del síndrome de Brugada

El síndrome de Brugada es una enfermedad hereditaria caracterizada por un patrón electrocardiográfico característico, así como por un aumento del riesgo de sufrir arritmias cardíacas y muerte súbita. No existen actualmente indicadores objetivos que permitan determinar el riesgo individual, por lo que la elegibilidad para la implantación de desfibriladores automáticos implantables con el fin de prevenir eventos de muerte súbita genera todavía gran controversia. Dado que el electrocardiograma (ECG) proporciona información cardíaca de forma no invasiva, se presenta un algoritmo automático capaz de detectar parámetros electrocardiográficos relevantes en la enfermedad. De este modo, futuros estudios que incluyan grandes series de pacientes permitirán determinar el valor pronóstico de las diferentes variables detectadas. El algoritmo integra funciones disponibles previamente desarrolladas para la lectura y delineación de señales ECG y propone una expansión centrada en la detección de parámetros como la carga de latidos con patrón de Brugada, elevación del segmento ST, duración del segmento ST y de los intervalos PR, QRS y QTc, la variabilidad del ritmo cardíaco (VRC), la alternancia de la onda T o la carga de extrasístoles ventriculares, entre otros. El código se testeó en registros ECG continuos de 12 derivaciones y 24 horas de duración de dos pacientes con diferentes grados de severidad y un caso control, obteniendo tendencias relacionadas con la VRC y la carga de latidos con patrón de Brugada. Asimismo, se analizaron diferentes colocaciones de los electrodos con el objetivo de concluir la configuración más sensible para la detección del patrón característico.Postprint (published version

Recent Advances in Short QT Syndrome

Short QT syndrome is a highly malignant inherited cardiac disease characterized by ventricular tachyarrhythmias leading to syncope and sudden cardiac death. It is responsible of lethal episodes in young people, mainly infants. International guidelines establish diagnostic criteria with the presence of a QTc ≤ 340 ms in the electrocardiogram despite clinical diagnostic values remain controversial. In last years, clinical diagnosis, risk stratification as well as preventive therapies have been improved due to identification of pathophysiological mechanisms. The only effective option is implantation of a defibrillator despite Quinidine may be at times an effective option. Currently, a limited number of rare variants have been identified in seven genes, which account for nearly 20–30% of families. However, some of these variants are associated with phenotypes showing a shorter QT interval but no conclusive diagnosis of Short QT syndrome. Therefore, an exhaustive interpretation of each variant and a close genotype-phenotype correlation is necessary before clinical translation. Here, we review the main clinical and genetic hallmarks of this rare entity

Outcomes of conduction system pacing compared to right ventricular pacing as a primary strategy for treating bradyarrhythmia: systematic review and meta-analysis

Background Right ventricular pacing (RVP) may cause electrical and mechanical desynchrony leading to impaired left ventricular ejection fraction (LVEF). We investigated the outcomes of RVP with His bundle pacing (HBP) and left bundle branch pacing (LBBP) for patients requiring a de novo permanent pacemaker (PPM) for bradyarrhythmia. Methods and results Systematic review of randomized clinical trials and observational studies comparing HBP or LBP with RVP for de novo PPM implantation between 01 January 2013 and 17 November 2020 was performed. Random and fixed effects meta-analyses of the effect of pacing technology on outcomes were performed. Study outcomes included all-cause mortality, heart failure hospitalization (HFH), LVEF, QRS duration, lead revision, atrial fibrillation, procedure parameters, and pacing metrics. Overall, 9 studies were included (6 observational, 3 randomised). HBP compared with RVP was associated with decreased HFH (risk ratio [RR] 0.68, 95% confidence interval [CI] 0.49–0.94), preservation of LVEF (mean difference [MD] 0.81, 95% CI − 1.23 to 2.85 vs. − 5.72, 95% CI − 7.64 to -3.79), increased procedure duration (MD 15.17 min, 95% CI 11.30–19.04), and increased lead revisions (RR 5.83, 95% CI 2.17–15.70, p = 0.0005). LBBP compared with RVP was associated with shorter paced QRS durations (MD 5.6 ms, 95% CI − 6.4 to 17.6) vs. (51.0 ms, 95% CI 39.2–62.9) and increased procedure duration (MD 37.78 min, 95% CI 20.04–55.51). Conclusion Of the limited studies published, this meta-analysis found that HBP and LBBP were superior to RVP in maintaining physiological ventricular activation as an initial pacing strategy

Impact of centre volume on atrial fibrillation ablation outcomes in Europe: a report from the ESC EHRA EORP Atrial Fibrillation Ablation Long-Term (AFA LT) Registry

Abstract Aims  The aim of the study was to investigate differences in clinical outcomes and complication rates among European atrial fibrillation (AF) ablation centres related to the volume of AF ablations performed. Methods and results  Data for this analysis were extracted from the ESC EHRA EORP European AF Ablation Long-Term Study Registry. Based on 33rd and 67th percentiles of number of AF ablations performed, the participating centres were classified into high volume (HV) (≥ 180 procedures/year), medium volume (MV) (<180 and ≥74/year), and low volume (LV) (<74/year). A total of 91 centres in 26 European countries enrolled in 3368 patients. There was a significantly higher reporting of cardiovascular complications and stroke incidence in LV centres compared with HV and MV (P = 0.039 and 0.008, respectively) and a lower success rate after AF ablation (55.3% in HV vs. 57.2% in LV vs. 67.4% in MV centres, P < 0.001), despite lower CHA2DS2-VASc score of patients, enrolled in LVs and less complex ablation techniques used. Adjustments of confounding factors (including type of AF ablation) led to elimination of these differences. Conclusion  Low-volume centres tended to present slightly higher cardiovascular complications' and stroke incidence and a lower unadjusted success rate after AF ablation, despite the fact that ablation procedures and patients were of lower risk compared with MV and HV centres. On the other hand, adjusted overall complication and recurrence rates were non-significantly different among different volume centres, a fact reflecting the heterogeneity of patient and procedural profiles, and a counterbalance between expertise and risk level among participating centres

Late gadolinium enhancement‐MRI determines definite lesion formation most accurately at 3 months post ablation compared to later time points

Aims: Neither the long-term development of ablation lesions nor the capability of late gadolinium enhancement (LGE)-MRI to detect ablation-induced fibrosis at late stages of scar formation have been defined. We sought to assess the development of atrial ablation lesions over time using LGE-MRI and invasive electroanatomical mapping (EAM). Methods and results: Ablation lesions and total atrial fibrosis were assessed in serial LGE-MRI scans 3 months and >12 months post pulmonary vein (PV) isolation. High-density EAM performed in subsequent repeat ablation procedures served as a reference. Serial LGE-MRI of 22 patients were analyzed retrospectively. The PV encircling ablation lines displayed an average LGE, indicative of ablation-induced fibrosis, of 91.7% ± 7.0% of the circumference at 3 months, but only 62.8% ± 25.0% at a median of 28 months post ablation (p 12 months post ablation. Accordingly, the agreement with EAM regarding detection of ablation-induced fibrosis and functional gaps was good for the LGE-MRI at 3 months (κ .74; p < .0001), but only weak for the LGE-MRI at 28 months post-ablation (κ .29; p < .0001). Conclusion: While non-invasive lesion assessment with LGE-MRI 3 months post ablation provides accurate guidance for future redo-procedures, detectability of atrial ablation lesions appears to decrease over time. Thus, it should be considered to perform LGE-MRI 3 months post-ablation rather than at later time-points > 12 months post ablation, like for example, prior to a planned redo-ablation procedure

BAG3 Genetic Cardiomyopathy May Overlap Fulminant Myocarditis Clinical Findings.

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Update on Genes Associated with Arrhythmogenic Cardiomyopathy

Arrhythmogenic cardiomyopathy is a rare genetic entity characterized by progressive fibro-fatty replacement of myocardium leading to malignant arrhythmias, syncope, and sudden cardiac death. Mostly it affects the right ventricle, but cases have also been described with biventricular and even isolated left ventricular involvement. The disease affects mainly young males and arrhythmias are usually induced by exercise. Arrhythmogenic cardiomyopathy has a genetic origin and is basically caused by deleterious alterations in genes encoding desmosomal proteins, especially plakophilin-2. To date, more than 400 rare genetic alterations have been identified in 18 genes, mainly with autosomal dominant inheritance, but some recessive forms have also been reported (Naxos disease and Carvajal syndrome). A comprehensive genetic analysis identifies a rare variant as potential cause of the disease in around 60% of patients, suggesting the existence of unknown genes as well as other genome alterations not yet discovered. Genetic interpretation classifies some of these rare variants as ambiguous, playing an uncertain role in arrhythmogenic cardiomyopathy. This makes a proper translation of genetic data into clinical practice difficult. Moreover, incomplete penetrance and variable phenotypic expression makes it difficult to arrive at the correct diagnosis. In the present chapter, we focus on recent advances in the knowledge regarding the genetic basis of arrhythmogenic cardiomyopathy