2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

X-ray management in electrophysiology: a survey of the Italian Association of Arrhythmology and Cardiac Pacing (AIAC).

AIMS: Radiation use in medicine has significantly increased over the last decade, and cardiologists are among the specialists most responsible for X-ray exposure. The present study investigates a broad range of aspects, from specific European Union directives to general practical principles, related to radiation management among a national cohort of cardiologists. METHODS AND RESULTS: A voluntary 31-question survey was run on the Italian Arrhythmology and Pacing Society (AIAC) website. From June 2019 to January 2020, 125 cardiologists, routinely performing interventional electrophysiology, participated in the survey. Eighty-seven (70.2%) participants are aware of the recent European Directive (Euratom 2013/59), although only 35 (28.2%) declare to have read the document in detail. Ninety-six (77.4%) participants register the dose delivered to the patient in each procedure, in 66.1% of the cases both as fluoroscopy time and dose area product. Years of exposition (P = 0.009) and working in centers performing pediatric procedures (P = 0.021) related to greater degree of X-ray equipment optimization. The majority of participants (72, 58.1%) did not recently attend radioprotection courses. The latter is related to increased awareness of techniques to reduce radiation exposure (96% vs. 81%, P = 0.022), registration of the delivered dose in each procedure (92% vs. 67%, P = 0.009), and X-ray equipment optimization (50% vs. 36%, P = 0.006). CONCLUSION: Italian interventional cardiologists show an acceptable level of radiation awareness and knowledge of updated European directives. However, there is clear space for improvement. Comparison to other health professionals, both at national and international levels, is needed to pursue proper X-ray management and protect public health.

2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias.

Cardiac surgeon and electrophysiologist shoulder-to-shoulder approach: Hybrid room, a kingdom for two. A zero mortality transvenous lead extraction single center experience.

BACKGROUND: Nowadays, transvenous lead extraction (TLE) is considered an essential technique in lead management strategy. Since 2011, a multidisciplinary approach was undertaken in our centre involving electrophysiologists, cardiac surgeons and anaesthesiologists to improve cross- unit cooperation and minimize complications and mortality. The present paper reports procedural outcomes and complications of our lead extraction experience. METHODS: We retrospectively collected and analysed data from all consecutive patients undergoing cardiac implantable electronic device leads TLE at the IRCCS Centro Cardiologico Monzino between January 2011 and November 2017. RESULTS: One-hundred fifty patients (111 males, 68 ±â€¯13 years) underwent extraction procedures. The most common extraction indication were infections (86.7%) and TLE was carried out by laser-based approach in 88 (58.6%) patients, by mechanical dilating sheaths in 58 (38.7%) patients and by a combined approach (TLE + open surgical intervention) in 4 (2.7%) patients. Procedural success was obtained in 146 (97.3%) cases with only 3 (2.0%) major complications with 2 cases of structural injury with tamponade requiring emergent median sternotomy. Open surgery extraction was required in 4 patients, after an attempt to TLE, due to leads strict adhesion to cardiac or vascular structures, whereas in 5 (3.3%) cases, the treatment of choice was a combined approach consisting in transvenous leads extraction followed by planned surgery. CONCLUSIONS: TLE is a complex procedure that sometimes leads to fatal complications. In our single center experience, a multidisciplinary approach involving electrophysiologist, cardiac surgeon, anaesthesiologist in an operating room allows a safer approach and major complications treatment.

QT interval variability in body surface ECG: measurement, physiological basis, and clinical value: position statement and consensus guidance endorsed by the European Heart Rhythm Association jointly with the ESC Working Group on Cardiac Cellular Electrophysiology.

This consensus guideline discusses the electrocardiographic phenomenon of beat-to-beat QT interval variability (QTV) on surface electrocardiograms. The text covers measurement principles, physiological basis, and clinical value of QTV. Technical considerations include QT interval measurement and the relation between QTV and heart rate variability. Research frontiers of QTV include understanding of QTV physiology, systematic evaluation of the link between QTV and direct measures of neural activity, modelling of the QTV dependence on the variability of other physiological variables, distinction between QTV and general T wave shape variability, and assessing of the QTV utility for guiding therapy. Increased QTV appears to be a risk marker of arrhythmic and cardiovascular death. It remains to be established whether it can guide therapy alone or in combination with other risk factors. QT interval variability has a possible role in non-invasive assessment of tonic sympathetic activity.

Registro Español de Desfibrilador Automático Implantable. XI Informe Oficial de la Sección de Electrofisiología y Arritmias de la Sociedad Española de Cardiología (2014) / Spanish Implantable Cardioverter-defibrillator Registry. Eleventh Official Report of the Spanish Society of Cardiology Electrophysiology and Arrhythmias Section (2014)

Introducción y objetivos. Se presentan los resultados del Registro Español de Desfibrilador Automático Implantable de 2014, elaborado por la Sección de Electrofisiología y Arritmias de la Sociedad Española de Cardiología. Métodos. Se envió de forma prospectiva a la Sociedad Española de Cardiología la hoja de recogida de datos cumplimentada voluntariamente por cada equipo implantador. Resultados. El número de implantes comunicados fue 4.911 (el 82% del total de implantes estimado). La tasa de implantes fue 106 por millón de habitantes y la estimada, 128. Los primoimplantes fueron el 72,2%. Se obtuvieron datos de 162 hospitales (8 más que en 2013). La mayoría de los implantes (82%) se realizaron en varones. La media de edad fue 61,8 ± 13,7 años. La mayoría de los pacientes presentaban una disfunción ventricular grave o grave-moderada y clase funcional II de la New York Heart Association. La cardiopatía más frecuente fue la isquémica, seguida de la dilatada. Las indicaciones por prevención primaria han sido el 58,5%. Los implantes realizados por electrofisiólogos fueron el 85,6%. Conclusiones. El Registro Español de Desfibrilador Automático Implantable de 2014 recoge información del 82% de los implantes realizados en España. El número de implantes ha crecido respecto a los datos de los últimos años. El porcentaje de indicación por prevención primaria ha aumentado con respecto al registro anterior (AU)

Introduction and objectives. We present the results of the Spanish Implantable Cardioverter-defibrillator Registry for 2014, as compiled by the Electrophysiology and Arrhythmia Section of the Spanish Society of Cardiology. Methods. Data collection sheets were voluntarily completed by each implantation team and prospectively sent to the Spanish Society of Cardiology. Results. The number of reported implantations was 4911 (82% of the estimated total number of implantations). The implantation rate was 106 per million population while the estimated rate was 128. First implantations comprised 72.2%. Data were obtained from 162 hospitals (8 more than in 2013). Most implantations (82%) were performed in men. The mean patient age was 61.8 ± 13.7 years. Most patients showed severe or moderate-to-severe ventricular dysfunction and were in New York Heart Association functional class II. The most frequent cardiac condition was ischemic heart disease, followed by dilated cardiomyopathy. Implantations for primary prevention indications comprised 58.5%. Electrophysiologists performed 85.6% of the implantations. Conclusions. The 2014 Spanish Implantable Cardioverter-defibrillator Registry received information on 82% of the implantations performed in Spain. The number of implantations has increased from previous years and the percentage of implantations for primary prevention indications has increased from the previous year (AU)

Registro Español de Trasplante Cardiaco. XXVI Informe Oficial de la Sección de Insuficiencia Cardiaca y Trasplante Cardiaco de la Sociedad Española de Cardiología (1984-2014) / Spanish Heart Transplantation Registry. 26th Official Report of the Spanish Society of Cardiology Working Group on Heart Failure and Heart Transplantation (1984-2014)

Introducción y objetivos. Se presentan las características y resultados del trasplante cardiaco en España desde que empezó su actividad en mayo de 1984. Métodos. Se realiza un análisis descriptivo de las características de los receptores, los donantes, el procedimiento quirúrgico y los resultados de los trasplantes cardiacos realizados en España hasta el 31 de diciembre de 2014. Resultados. Durante 2014 se han realizado 266 procedimientos, con lo que la serie histórica consta de 7.289 trasplantes. El análisis temporal confirma un empeoramiento significativo del perfil clínico de los receptores (mayor porcentaje de pacientes añosos, con insuficiencia renal grave, diabetes insulinodependiente, cirugía cardiaca previa y ventilación mecánica), de los donantes (mayor porcentaje de donantes añosos y con mayor discordancia de peso) y del procedimiento (mayor porcentaje de trasplante urgente, que en 2014 alcanza el 41,4%, y con tiempo de isquemia > 240 min). El uso de dispositivos de asistencia mecánica ha disminuido respecto a 2013, y en 2014 supone el 18,8% del total de pacientes. La supervivencia a 1, 5, 10 y 15 años es del 76, el 65, el 52 y el 38% respectivamente, y permanece estable desde 1995. Conclusiones. La actividad de trasplante cardiaco en España permanece estable en los últimos años, con alrededor de 250 procedimientos al año. A pesar del claro empeoramiento de las características de donantes, receptores y tiempos quirúrgicos, se mantienen unos resultados en mortalidad comparables a los del entorno y se confirma un uso creciente de los dispositivos de asistencia circulatoria antes del trasplante (AU)

Introduction and objectives. We present the characteristics and outcomes of heart transplantation in Spain since it was first performed in 1984. Methods. A descriptive analysis of the characteristics of recipients, donors, the surgical procedure, and the outcomes of heart transplantations performed in Spain until 31 December 2014. Results. In 2014, 266 procedures were performed, making a time series of 7289 transplantations. The temporal analysis confirmed a significant worsening of the clinical profile of recipients (higher percentage of older patients, patients with severe renal failure, insulin-dependent diabetes, previous cardiac surgery, and previous mechanical ventilation), of donors (higher percentage of older donors and greater weight mismatch), and of the procedure (higher percentage of emergency transplantations, reaching 41.4% in 2014, and ischemia time > 240 min). Mechanical assist devices were used less than in 2013; in 2014 they were used in 18.8% of all transplant recipients. Survival at 1, 5, 10, and 15 years was 76%, 65%, 52%, and 38%, respectively, and has remained stable since 1995. Conclusions. Cardiac transplantation activity in Spain has remained stable in recent years, at around 250 procedures per year. Despite a clear deterioration in donor and recipient characteristics and surgical times, the mortality outcomes have remained comparable to those of previous periods in our environment. The growing use of circulatory assist devices before transplantation is also confirmed (AU)

Guías para el manejo de los desfibriladores automáticos implantables en las fases finales de la vida / Guidelines on the management of implantable cardioverter-defibrillators at the end of life

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Opportunities and challenges of current electrophysiology research: a plea to establish 'translational electrophysiology' curricula.

Cardiac electrophysiology has evolved into an important subspecialty in cardiovascular medicine. This is in part due to the significant advances made in our understanding and treatment of heart rhythm disorders following more than a century of scientific discoveries and research. More recently, the rapid development of technology in cellular electrophysiology, molecular biology, genetics, computer modelling, and imaging have led to the exponential growth of knowledge in basic cardiac electrophysiology. The paradigm of evidence-based medicine has led to a more comprehensive decision-making process and most likely to improved outcomes in many patients. However, implementing relevant basic research knowledge in a system of evidence-based medicine appears to be challenging. Furthermore, the current economic climate and the restricted nature of research funding call for improved efficiency of translation from basic discoveries to healthcare delivery. Here, we aim to (i) appraise the broad challenges of translational research in cardiac electrophysiology, (ii) highlight the need for improved strategies in the training of translational electrophysiologists, and (iii) discuss steps towards building a favourable translational research environment and culture.

Periaortitis fibrosa con infiltración en las válvulas aórtica y mitral / Fibrotic periaortitis infiltrating into the aortic and mitral valves

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Diagnóstico diferencial entre doble respuesta ventricular y extrasistolia hisiana bigeminada. Respuesta / Differential diagnosis between dual ventricular response and bigeminy arising from the bundle of his. Response

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Evaluación de la complejidad de la activación miocárdica durante la fibrilación ventricular. Estudio experimental / Evaluation of the complexity of myocardial activation during ventricular fibrillation. An experimental study

Introducción y objetivos. Analizar en un modelo experimental las características de la fibrilación ventricular en situaciones con distintos grados de complejidad y establecer la relación existente entre los datos aportados por distintos métodos de análisis de la arritmia. Métodos. En 27 preparaciones de corazón aislado de conejo estudiadas bajo la acción de fármacos (propranolol y KB-R7943) o procedimientos físicos (estiramiento) que causan distintos grados de variación de la complejidad de la activación miocárdica durante la arritmia, se han utilizado técnicas espectrales, morfológicas y cartográficas para procesar los registros obtenidos con multielectrodos epicárdicos. Resultados. La complejidad de la fibrilación ventricular objetivada mediante procedimientos cartográficos se ha relacionado con la frecuencia dominante, la energía normalizada del espectro, el índice de regularidad de las señales, sus coeficientes de variación y el área de las regiones de interés identificadas a partir de estos parámetros. En el análisis multivariable, se han aceptado como variables independientes el área de las regiones de interés relacionadas con la energía espectral y el coeficiente de variación de la energía (índice de complejidad = -0,005 × área de las regiones de la energía espectral -2,234 × coeficiente de variación de la energía +1,578; p = 0,0001; r = 0,68). Conclusiones. Los indicadores espectrales, morfológicos y, de manera independiente, los derivados del análisis de las regiones de interés de la energía normalizada permiten aproximarse de manera fiable a la evaluación de la complejidad de la fibrilación ventricular como una alternativa a los complejos procedimientos cartográficos (AU)

Introduction and objectives. An experimental model is used to analyze the characteristics of ventricular fibrillation in situations of variable complexity, establishing relationships among the data produced by different methods for analyzing the arrhythmia. Methods. In 27 isolated rabbit heart preparations studied under the action of drugs (propranolol and KB-R7943) or physical procedures (stretching) that produce different degrees of change in the complexity of myocardial activation during ventricular fibrillation, use was made of spectral, morphological, and mapping techniques to process the recordings obtained with epicardial multielectrodes. Results. The complexity of ventricular fibrillation assessed by mapping techniques was related to the dominant frequency, normalized spectral energy, signal regularity index, and their corresponding coefficients of variation, as well as the area of the regions of interest identified on the basis of these parameters. In the multivariate analysis, we used as independent variables the area of the regions of interest related to the spectral energy and the coefficient of variation of the energy (complexity index = -0.005 × area of the spectral energy regions -2.234 × coefficient of variation of the energy +1.578; P=.0001; r=0.68). Conclusions. The spectral and morphological indicators and, independently, those derived from the analysis of normalized energy regions of interest provide a reliable approach to the evaluation of the complexity of ventricular fibrillation as an alternative to complex mapping techniques (AU)

Manifestaciones cardiacas en los pacientes con distrofia miotónica tipo 1 seguidos de forma protocolizada en una consulta monográfica / Cardiac manifestations in myotonic dystrophy type 1 patients followed using a standard protocol in a specialized unit

Introducción y objetivos. La distrofia miotónica tipo 1 se caracteriza por afección muscular y manifestaciones sistémicas, entre ellas las cardiacas. Nuestro objetivo es documentar la frecuencia y la gravedad de la afección cardiovascular (aparición de disfunción ventricular izquierda y trastornos del ritmo o conducción), la necesidad de implantar marcapasos o desfibrilador o de realizar estudio electrofisiológico y la aparición de muerte súbita durante el seguimiento. Métodos. Estudio observacional retrospectivo de los pacientes con distrofia miotónica tipo 1 remitidos a una consulta monográfica de cardiología y sometidos a seguimiento clínico, electrocardiográfico (con registro Holter) y ecocardiográfico. Resultados. Se incluyó a 81 pacientes (el 51,9% varones; media de edad, 29,9 ± 14,8 años). El seguimiento medio fue de 5,7 ± 3,9 (1-20) años y se documentó bradicardia sinusal en el 48,8%, disfunción sinusal en el 13,8%, arritmias supraventriculares en el 10%, intervalo PR ≥ 220 ms en el 31,3%, taquicardia ventricular el 5%, intervalo QT corregido largo en el 5%, bloqueo auriculoventricular de segundo o tercer grado en el 8,8% e intervalo QRS ≥ 120 ms en el 7,5%. Sólo 1 paciente presentó disfunción ventricular grave. Durante el seguimiento se implantaron 15 marcapasos y 2 desfibribladores y se realizaron 5 estudios electrofisiológicos, la mayoría por taquicardia ventricular. Sólo se produjo 1 muerte súbita. Conclusiones. Los trastornos de conducción y del ritmo son frecuentes durante la evolución de dichos pacientes, y un porcentaje considerable requiere estudio electrofisiológico e implante de dispositivos (marcapasos o desfibrilador). La disfunción sistólica y la muerte súbita son excepcionales en nuestra experiencia (AU)

Introduction and objectives. Myotonic dystrophy type 1 is characterized by muscle damage and systemic manifestations, including cardiac involvement. Our aim was to document the frequency and severity of cardiac involvement (left ventricular dysfunction and arrhythmia or conduction disorders), the need for a pacemaker, implantable cardioverter-defibrillator, or electrophysiological study, and the development of sudden death during follow-up. Methods. Retrospective observational study of myotonic dystrophy type 1 patients referred to a specialized cardiac unit. Patients received clinical, electrocardiographic (Holter monitoring), and echocardiographic follow-up. Results. We included 81 patients (51.9% men; mean age, 29.9 [14.8] years). The mean follow-up was 5.7 (3.9) years (range: 1-20 years). During this period sinus bradycardia was documented in 48.8%, PR interval≥220 ms in 31.3%, long corrected QT interval in 5%, and QRS interval≥120 ms in 7.5%. A total of 13.8% of patients developed sinus node dysfunction, 10% of patients had supraventricular arrhythmias, 5% had ventricular tachycardia, and 8.8% developed second- or third- degree atrioventricular block. Only 1 patient had severe ventricular dysfunction. During the follow-up, 15 pacemakers and 2 implantable cardioverter-defibrillators were implanted and 5 electrophysiological studies were performed (mainly due to ventricular tachycardia). There was only 1 sudden death. Conclusions. Arrhythmia or conduction disorders are frequent during the course of myotonic dystrophy type 1 patients. A significant percentage of patients require electrophysiological study and the use of a device (pacemaker or implantable cardioverter-defibrillator). In our experience, systolic dysfunction and sudden death are rare (AU)

Novedades en alteraciones del ritmo cardiaco: electrofisiología cardiaca, arritmias y estimulación cardiaca / Innovations in heart rhythm disturbances: cardiac electrophysiology, arrhythmias, and cardiac pacing

En este artículo se revisan los trabajos más relevantes en el campo de las arritmias publicados en 2012, incluyendo aritmología clínica, técnicas de ablación, estimulación cardiaca y genética de la muerte súbita (AU)

This article reviews the most relevant articles published in 2012 in the field of arrhythmias, on subjects that include clinical arrhythmology, ablation, cardiac pacing, and the genetics of sudden cardiac death (AU)

Doble respuesta ventricular: otra vía a la taquicardia supraventricular en fisiología doble del nódulo / Dual ventricular response: another road to supraventricular tachycardia in dual atrioventricular nodal physiology

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[Changes of QT interval during graded exercise test in healthy adolescents aged 11-15 years].

Aim of the study was determination of physiological limits of QT-intervals and its derivative values in healthy children and adolescents during graded exercise tests. We examined 100 healthy boys and girls aged 11-15 years (mean age 13.4+/-2.1 years) and performed electrocardiography at rest and standard veloergometry (VEM) in all of them. We analyzed corrected intervals according to Bazett (QTc=QT/RR) and Fredericia (FQTc/3RR) formulas. Hysteresis QTc was calculated as difference between QTc durations during recovery and exercise at same heart rate (HR) Baseline HR before VEM exceeded rhythm on resting electrocardiogram by 5-15 bpm (84+/-8 vs 70+/-6, respectively, p<0.05) Increase of HR at exercise (mean 172+/-11 bpm) was similar in both sexes. QT interval decreased by 7-10% (18-31 ms) per each 25 w (p<0.05). Values obtained at determination of FQTc we found values 26-52 ms lower than those calculated by the Bazett formula in the process of whole test. Determination of FQTc compared with calculation by Bazett formula revealed more pronounced (10% from baseline level) shortening of FQT at peak exercise. QT calculated by the Bazett formula at 100 w did not differ from baseline level with tendency to higher level. Corrected QT according to the most often used Bazett formula was maximal at the first stage of exercise (25 w) and did not exceed 450 ms in boys and 460ms in girls. Maximal QTc lengthening in the process of test did not exceed 50 ms in any of the examined persons. Hysteresis of QTc interval was equal to 21+/-6 (15-25) ms. The conclusion was made that algorithm of assessment of QT interval changes during exercise test should include initial values of QTc calculated according to the Bazett formula, maximal QTc value, level of exercise at which it was registered, maximal increase of QTc during exercise, and QTc interval hysteresis.