Uma Causa Rara e Tratamento da Fibrilação Ventricular: Toxicidade do 5-Fluorouracil / A Rare Cause and Management of Ventricular Fibrillation: 5-Fluorouracil Toxicity

Resumo Um homem de 65 anos com histórico de carcinoma de língua procurou o pronto-socorro com contrações insensíveis estando em casa. Ele estava em terapia com 5-fluorouracil (5-FU) na época. O paciente foi desfibrilado e intubado porque a fibrilação ventricular (FV) se desenvolveu durante o monitoramento no pronto-socorro. A ecocardiografia mostrou que a fração de ejeção do ventrículo esquerdo (FEVE) era de 70% e a espessura do septo interventricular era de 15 mm. A angiografia coronária não revelou qualquer estenose crítica. A ressonância magnética cardíaca (RMC) não mostrou anormalidade de perfusão, fibrose ou cicatriz sugestiva de envolvimento cardíaco. Foi sugerido que a arritmia do paciente estava relacionada principalmente à cardiotoxicidade induzida pelo 5-FU. O fato de as causas secundárias terem sido proeminentes em nosso caso, de nenhuma patologia cardíaca óbvia que pudesse causar arritmia ter sido encontrada no exame detalhado e de a arritmia não ter recorrido durante a internação hospitalar, que durou até 15 dias, nos levou a acreditar que esse paciente poderia receber alta sem um cardioversor-desfibrilador implantável. Nosso caso foi apresentado para contribuir com a literatura.

KCNH2 mutation c.3099_3112del causes congenital long QT syndrome type 2 with gender differences.

INTRODUCTION: Long QT Syndrome (LQTS) is an inherited disease with an abnormal electrical conduction system in the heart that can cause sudden death as a result of QT prolongation. LQT2 is the second most common subtype of LQTS caused by loss of function mutations in the potassium voltage-gated channel subfamily H member 2 (KCNH2) gene. Although more than 900 mutations are associated with the LQTS, many of these mutations are not validated or characterized. METHODS AND RESULTS: Sequencing analyses of genomic DNA of a family with LQT2 identified a putative mutation. i.e., KCNH2(NM_000238.3): c.3099_3112del, in KCNH2 gene which appeared to be a definite pathogenic mutation. The family pedigree information showed a gender difference in clinical features and T-wave morphology between male and female patients. The female with mutation exhibited recurring ventricular arrhythmia and syncope, while two male carriers did not show any symptoms. In addition, T-wave in females was much flatter than in males. The female proband showed a positive reaction to the lidocaine test. Lidocaine injection almost completely blocked ventricular arrhythmia and shortened the QT interval by ≥30 ms. Treatment with propranolol, mexiletine, and implantation of cardioverter-defibrillators prevented the sustained ventricular tachycardia, ventricular fibrillation, and syncope, as assessed by a 3-year follow-up evaluation. CONCLUSIONS: A putative mutation c.3099_3112del in the KCNH2 gene causes LQT2 syndrome, and the pathogenic mutation mainly causes symptoms in female progeny.

Ventricular Fibrillation Is a Sign of Life.

Ventricular fibrillation (VF) is a deadly rhythm problem. With asystole, it represents one of the most extreme emergencies that may engage vital prognosis within only few minutes if appropriated treatment is not instituted. It is learned in all medical schools worldwide that VF is not compatible with consciousness and sustained life. Moreover, at 37°C, and without restauration of cardiac flow, VF may be responsible for severe and most often irreversible brain damage after 3 minutes.

Different Ventricular Fibrillation Types in Low-Dimensional Latent Spaces.

The causes of ventricular fibrillation (VF) are not yet elucidated, and it has been proposed that different mechanisms might exist. Moreover, conventional analysis methods do not seem to provide time or frequency domain features that allow for recognition of different VF patterns in electrode-recorded biopotentials. The present work aims to determine whether low-dimensional latent spaces could exhibit discriminative features for different mechanisms or conditions during VF episodes. For this purpose, manifold learning using autoencoder neural networks was analyzed based on surface ECG recordings. The recordings covered the onset of the VF episode as well as the next 6 min, and comprised an experimental database based on an animal model with five situations, including control, drug intervention (amiodarone, diltiazem, and flecainide), and autonomic nervous system blockade. The results show that latent spaces from unsupervised and supervised learning schemes yielded moderate though quite noticeable separability among the different types of VF according to their type or intervention. In particular, unsupervised schemes reached a multi-class classification accuracy of 66%, while supervised schemes improved the separability of the generated latent spaces, providing a classification accuracy of up to 74%. Thus, we conclude that manifold learning schemes can provide a valuable tool for studying different types of VF while working in low-dimensional latent spaces, as the machine-learning generated features exhibit separability among different VF types. This study confirms that latent variables are better VF descriptors than conventional time or domain features, making this technique useful in current VF research on elucidation of the underlying VF mechanisms.

Síndrome de Brugada: 30 Anos de Aventura Científica / Brugada Syndrome: 30 Years of Scientific Ventures

Resumo Trinta anos atrás, uma nova síndrome clínico-eletrocardiográfica distinta foi descrita, agora conhecida como síndrome de Brugada (SBr). Típico para essa síndrome é o eletrocardiograma com supradesnivelamento do segmento ST nas derivações precordiais direitas. A apresentação clínica da doença é altamente variável: os pacientes podem permanecer completamente assintomáticos, mas também podem desenvolver episódios de síncope, fibrilação atrial (FA), síndrome do nódulo sinusal (SNS), distúrbios de condução, assistolia e fibrilação ventricular (FV). A doença é causada por mutações nos genes responsáveis pelo potencial de ação das células do coração. O gene mais frequentemente envolvido é o SCN5A, que controla a estrutura e função do canal de sódio cardíaco. A descrição dessa nova síndrome teve implicações muito positivas em todos os campos da medicina.

Abstract Thirty years ago, a distinctly new clinical-electrocardiographic syndrome was described, today known as Brugada Syndrome (BrS). Typical treatment for this type of syndrome is electrocardiography with ST-segment elevation in the direct precordial derivations. The clinical presentation of the disease is highly variable: the patients can remain completely asymptomatic, but they can also develop episodes of syncope, atrial fibrillation (AF), sinus node dysfunction (SNF), conduction disorders, asystole, and ventricular fibrillation (VF). This disease is caused by mutations in the genes responsible for the potential action of cardiac cells. The most commonly involved gene is SCN5A, which controls the structure and function of the heart's sodium channel. The description of this new syndrome has shown highly positive implications in all fields of medicine.

Ventricular Fibrillation Is a Sign of Life

ABSTRACT Ventricular fibrillation (VF) is a deadly rhythm problem. With asystole, it represents one of the most extreme emergencies that may engage vital prognosis within only few minutes if appropriated treatment is not instituted. It is learned in all medical schools worldwide that VF is not compatible with consciousness and sustained life. Moreover, at 37°C, and without restauration of cardiac flow, VF may be responsible for severe and most often irreversible brain damage after 3 minutes.

KCNH2 mutation c.3099_3112del causes congenital long QT syndrome type 2 with gender differences

Abstract Introduction: Long QT Syndrome (LQTS) is an inherited disease with an abnormal electrical conduction system in the heart that can cause sudden death as a result of QT prolongation. LQT2 is the second most common subtype of LQTS caused by loss of function mutations in the potassium voltage-gated channel subfamily H member 2 (KCNH2) gene. Although more than 900 mutations are associated with the LQTS, many of these mutations are not validated or characterized. Methods and results: Sequencing analyses of genomic DNA of a family with LQT2 identified a putative mutation. i.e., KCNH2(NM_000238.3): c.3099_3112del, in KCNH2 gene which appeared to be a definite pathogenic mutation. The family pedigree information showed a gender difference in clinical features and T-wave morphology between male and female patients. The female with mutation exhibited recurring ventricular arrhythmia and syncope, while two male carriers did not show any symptoms. In addition, T-wave in females was much flatter than in males. The female proband showed a positive reaction to the lidocaine test. Lidocaine injection almost completely blocked ventricular arrhythmia and shortened the QT interval by ≥30 ms. Treatment with propranolol, mexiletine, and implantation of cardioverter-defibrillators prevented the sustained ventricular tachycardia, ventricular fibrillation, and syncope, as assessed by a 3-year follow-up evaluation. Conclusions: A putative mutation c.3099_3112del in the KCNH2 gene causes LQT2 syndrome, and the pathogenic mutation mainly causes symptoms in female progeny.

Miocardiopatía arritmogénica. Genes y proteínas desmosómicas / Arrhythmogenic Cardiomyopathy. Genes and Desmosomal Proteins

RESUMEN Desde 1996 esta enfermedad figura en la clasificación de las miocardiopatías de la OMS con el nombre de "miocardiopatía arritmogénica". A fines de la década del 70 se estableció que el ventrículo derecho (VD) puede ser el sustrato para el desarrollo de arritmias. En la década del 80 se describió el reemplazo del miocardio por tejido fibroadiposo y su naturaleza hereditaria. Posteriores descubrimientos permitieron la identificación de varios genes implicados en la producción de proteínas desmosómicas que participan en el acoplamiento intercelular lo cual llevó a definir a la miocardiopatía arritmogénica como una enfermedad desmosómica. El electrocardiograma y el ecocardiograma resultaron fundamentales y la angiocardiografía invasiva se utilizó para detectar disquinesia-aquinesia y aneurismas del VD. La biopsia endomiocárdica se perfiló como el gold standard para el diagnóstico, debido a su capacidad para detectar el reemplazo transmural por tejido fibroadiposo. El advenimiento de la resonancia magnética cardíaca (RMC) con realce tardío de gadolinio ha permitido revelar no solamente anomalías morfológico-funcionales sino también daño tisular. El conocimiento de la estructura del disco intercalar, involucrado en el acoplamiento intercelular ha permitido determinar que no solamente los desmosomas estarían comprometidos, sino que habría varias proteínas constituyentes tanto de los desmosomas, como de las uniones adherentes, las uniones gap, y los canales iónicos, integradas en una unidad conocida como "área composita". Ésta constituye una amalgama entre elementos de sostén y canales iónicos que participan en la propagación del potencial de acción, lo que ha permitido desarrollar el concepto de disco intercalar compuesto por los llamados "nodos excitoadhesivos". Las implicancias clínicas en el desarrollo de arritmias malignas son obvias.

ABSTRACT In 1996 this disease was introduced into the WHO classification of cardiomyopathies with the term "arrhythmogenic cardiomyopathy". By the end of the 70s the right ventricle (RV) was identified as a substrate for the development of arrhythmias. The replacement of the myocardium by fibrofatty tissue and the hereditary nature of this condition were described in the 1980s. Later findings led to the identification of several genes involved in the production of desmosomal proteins participating in intercellular coupling, which led to defining arrhythmogenic cardiomyopathy as a desmosomal disease. Electrocardiography and echocardiography are fundamental tools, and invasive angiocardiography was used to detect dyskinesia-akinesia and right ventricular aneurysms. Endomyocardial biopsy was established as the gold standard for the diagnosis due to its ability to detect transmural replacement by fibrofatty tissue. The advent of cardiac magnetic resonance imaging (CMRI) with late gadolinium enhancement reveals morphological and functional abnormalities and tissue damage. The understanding of intercalated disc structure involved in intercellular coupling has made it possible to determine that, apart from desmosomes, several desmosomal proteins, as adherens junctions, gap junctions and ion channels are integrated into a unit known as the " area composita". The area composita constitutes an amalgam between supporting elements and ion channels that participate in action potential propagation, which has led to develop the concept that intercalated discs are constituted by "adhesion/ excitability nodes". The clinical implications in the development of malignant arrhythmias are obvious.

Cardiodesfibrilador implantable: estado del arte / Cardiac defibrillators: state of the art

Resumen La muerte súbita cardiaca es una consecuencia devastadora de las enfermedades estructurales del corazón y un problema de salud pública en todo el mundo; es responsable de alrededor del 50% de las muertes por causa cardiovascular. Su incidencia es mayor en personas por encima de los de 40 años, siendo en esta población la cardiopatía isquémica instaurada o durante la fase aguda del infarto al miocardio los factores de riesgo más importantes; sin embargo, hay otros factores no relacionados con isquemia, como la cardiomiopatía dilatada, hipertrófica o valvular. La fibrilación y la taquicardia ventricular son la causa más frecuente de muerte súbita cardiaca en adultos. Los cardiodesfibriladores implantables son ampliamente utilizados y recomendados por las sociedades de cardiología para la prevención primaria y secundaria de la muerte súbita cardiaca.

Abstract Sudden cardiac death is a devastating consequence of structural heart disease and a global public health problem, accounting for close to 50% of cardiovascular deaths. Its incidence is greater in people over the age of 40, with the most important risk factors being: established ischemic heart disease or ischemia during the acute phase of a myocardial infarction. However, there are other factors, unrelated to ischemia, such as dilated, hypertrophic, or valvular cardiomyopathy. Ventricular fibrillation and tachycardia are the most frequent causes of sudden cardiac death in adults. Implantable cardioverter-defibrillators are widely used and recommended by cardiology societies for primary and secondary prevention of sudden cardiac death.

Disyunción del anillo mitral como marcador de riesgo en prolapso de válvula mitral / The mitral annulus disjunction as a risk marker in mitral valve prolapse

Resumen La disyunción del anillo valvular mitral es una anormalidad estructural que consiste en un desplazamiento auricular del punto de articulación de la válvula mitral, lo que lleva a una relación espacial alterada entre la válvula y la pared ventricular posterior adyacente. Los estudios han demostrado una relación entre la disyunción del anillo mitral, las arritmias ventriculares y la degeneración fibrosa miocárdica, lo que incrementa el riesgo de muerte súbita, en especial en mujeres jóvenes. Se presenta el caso de una mujer de 30 años, sin antecedentes relevantes, con palpitaciones frecuentes, en quien se documentó un aumento del automatismo ventricular con incremento progresivo de la carga arrítmica hasta el 20% a pesar del manejo farmacológico. En la resonancia cardiaca se apreció un desplazamiento de 15 mm hacia la aurícula de las valvas de la mitral compatible con disyunción del anillo mitral, además de focos de realce tardío de distribución mesocárdica, no coronariana. Ante los hallazgos y la persistencia de los síntomas, se llevó a estudio electrofisiológico, mapeo 3D y ablación. En la ecografía intracardiaca se apreciaron dos regiones hiperecoicas, en la longitud del músculo papilar anterior y en el mesocardio de la base del músculo papilar posterior; ambos focos relacionados con los sitios morfológicos de interés, en los cuales se aplicó energía de radiofrecuencia. Durante el procedimiento presentó un episodio de fibrilación ventricular que se consideró una extrasistolia ventricular maligna, por lo que se implantó un cardiodesfibrilador para prevención de muerte súbita. Se revisa la literatura y se analizan las relaciones fisiopatológicas existentes entre la disyunción del anillo mitral, los complejos ventriculares prematuros y el riesgo de muerte súbita.

Abstract Disjunction of the mitral valve annulus is a structural abnormality consisting of an atrial displacement of the articulation point of the mitral valve, leading to an altered spatial relationship between the valve and the adjacent posterior ventricular wall. Studies have shown the relationship between mitral annular disjunction, ventricular arrhythmias, and myocardial fibrous degeneration, which increases the risk of sudden death, especially among young women. The case of a 30-year-old woman with no relevant pathological history with frequent palpitations is presented, in whom an increase in ventricular automatism with a progressive increase in the arrhythmic load of up to 20% with a weight of pharmacological management is documented. In a cardiac magnetic resonance, a 15 mm displacement towards the atrium of the mitral leaflets compatible with mitral annular disjunction and late enhancement foci of mesocardial, non-coronary distribution were observed. Given the findings and the persistence of symptoms, an electrophysiological study, 3D mapping, and ablation were performed. Two hyperechoic regions were seen on intracardiac ultrasound, one in the length of the anterior papillary muscle and the other in the mesocardium of the base of the posterior papillary muscle. Both foci related to the morphological sites of interest in which radiofrequency energy was applied. During the procedure presented an episode of ventricular fibrillation. A malignant ventricular extrasystole was considered and therefore a cardio defibrillator was implanted for the secondary prevention of sudden death. The literature was reviewed and the specific pathophysiological relationships between mitral annular disjunction, premature ventricular complexes, and risk of sudden death were analyzed. The role of electrophysiological study and ablation in symptomatic patients refractory to pharmacological treatment is described.

Takotsubo Cardiomyopathy Associated with High-Grade Atrioventricular Block and Ventricular Fibrillation: A Case Report.

Takotsubo cardiomyopathy (TCM) is a condition characterized by reversible left ventricular dysfunction. TCM usually has a good prognosis but, in rare situations, it can be associated with life-threatening arrhythmias. We report a case and the management of TCM with a high-grade AV block and QT prolongation followed by ventricular fibrillation.

Muerte súbita cardíaca por fibrilación ventricular asociada a isquemia miocárdica, tratada por desfibrilador automático implantable / Sudden cardiac death due to ventricular fibrillation associated with myocardial ischemia, treated by implantable cardioverter defibrillator

Resumen: Reportamos el caso de un hombre de 67 años, con múltiples factores de riesgo cardiovascular, quien en el año 2015 presentó muerte súbita por fibrilación ventricular sin lesiones coronarias significativas, tras lo cual se implantó un desfibrilador automático. En el año 2019 presentó un nuevo episodio de fibrilación ventricular en relación con síndrome coronario agudo con supradesnivel del ST localizado en pared anterior, registrado y adecuadamente resuelto por el dispositivo. Se demostró una oclusión aterotrombótica en la porción proximal de la arteria descendente anterior. Fue precoz y exitosamente manejado con angioplastía coronaria percutánea e implante de stent fármaco activo, guiado por tomografía por coherencia óptica intracoronaria. A las 48 horas post angioplastía, presentó episodio de taquicardia ventricular polimorfa reconocido y tratado por el cardiodesfibrilador, sin consecuencias. Electivamente se efectuó angioplastía e implante de stent metálico en arteria coronaria derecha distal, con buen resultado angiográfico. La posterior evolución del paciente fue satisfactoria, sin manifestaciones de insuficiencia cardíaca, angina ni arritmias.

Abstract: A 67-year-old man, with multiple cardiovascular risk factors who in 2015 presented sudden death due to ventricular fibrillation without significant coronary lesions, after which an implantable automatic defibrillator (ICD) was implanted. In 2019, he presented a new episode of ventricular fibrillation adequately resolved by the device, in relation to an acute coronary syndrome with ST elevation in the anterior wall of the left ventricle, due to atherothrombotic occlusion in the proximal portion of the anterior descending artery. He was early and successfully managed with percutaneous coronary angioplasty by the insertion of a drug eluting stent, implanted guided by intracoronary optical coherence tomography. Forty eight hours later, he presented an episode polymorphic ventricular tachycardia recognized and treated by the ICD. Angioplasty with a bare metal stent implantation were performed in the distal right coronary artery, with good angiographic results. The subsequent course was satisfactory, with no manifestations of heart failure, angina or arrhythmias.

Intoxicação por Taxus baccata: sol após a tempestade elétrica / Taxus baccata intoxication: the sun after the electrical storm

RESUMO O teixo europeu (Taxus baccata) é uma árvore com pequenos ramos alternados, agulhas verdes e casca marrom-avermelhada. A ingestão de uma alta dose de Taxus baccata com intenção suicida geralmente resulta em morte. A toxicidade sistêmica é principalmente cardíaca. Os autores descrevem o caso de uma jovem paciente que ingeriu uma alta dose de agulhas de teixo e foi trazida ao pronto-socorro com intoxicação grave, manifestada por arritmias malignas caóticas, tratadas com sucesso após exaustivos cuidados de suporte.

ABSTRACT European yew (Taxus baccata) is a tree with alternate branchlets, green needles and reddish-brown bark. A high-dose ingestion of Taxus baccata for suicidal purposes usually results in death. The systemic toxicity is mainly cardiac. The authors describe the case of a young patient who ingested a high dose of yew needles and presented to the emergency department with a serious intoxication, which manifested as a chaotic malignant arrhythmia that was successfully treated after exhaustive supportive care.

Síndrome de brugada en el paciente quirúrgico de urgencia. Reporte de caso / Brugada syndrome in the emergency surgical patient. Case report.

Introducción: El síndrome de Brugada es una condición genética rara, el diagnóstico se establece por un patrón electrocardiográfico en particular que se asocia a un riesgo de fibrilación ventricular y muerte súbita; Objetivo: Presentar un caso interesante de un paciente joven quién ingreso a quirófano con la impresión clínica de apendicitis aguda para realizar apendicetomía video laparoscópica. Durante el procedimiento presento múltiples episodios de taquiarritmias; desencadenando Fibrilación Ventricular de difícil manejo trans y post operatorio, debido a los medicamentos peri-operatorios, agresión quirúrgica y fiebre. Material y Métodos: Se documento y presentó un caso interesante; Presentación de Caso: Paciente masculino de 26ª, sin antecedentes médicos, con impresión clínica de apendicitis aguda ingresa a quirófano, durante el acto quirúrgico presenta arritmias, documentándose Fibrilación Ventricular con inestabilidad hemodinámica que amerito desfibrilación externa, revirtió a ritmo sinusal. Al concluir el acto quirúrgico, se realiza EKG, evidenciando supradesnivel del segmento ST en V1 y V2 e inversión de la onda T por lo que pasa a unidad de cuidados intensivos, sin reversión anestésica y con tubo orotraqueal. Paciente se monitoriza en UTI, se realiza EKG evidencia ritmo nodal. Se extuba a las 12h post operatorias y se traslada a cardiología en 48h donde establecen que paciente cursa con patrón de Brugada. Conclusiones: Con el creciente número de pacientes con trastornos de conducción heredadas que se presentan para cirugía no cardiaca que están en riesgo de muerte súbita; el éxito en el manejo peri, trans y post operatorio depende de un conocimiento detallado de estas condiciones. (AU)

Introduction: Brugada syndrome is a rare genetic condition, the diagnosis is established by a particular electrocardiographic pattern and is associated with a risk of ventricular fibrillation and sudden death; Objective: To present an interesting case of a young patient who enters the operating room with the clinical impression of acute appendicitis to perform laparoscopic appendectomy and that during the procedure present multiple episodes of tachyarrhythmias; triggering Ventricular Fibrillation, what causes him difficult trans and postoperative management, due to peri-operative medications, surgical aggression and fever. Material and Methods: An interesting case was documented and presented; Case Presentation: Male patient of 26 years old, without medical history, with clinical impression of acute appendicitis was admitted to the operating room, during the surgical act it presents arrhythmias, showing Ventricular Fibrillation with hemodynamic instability that warrants external defibrillation, reversed at sinus pace, EKG is performed showing elevation gain of the ST segment in V1 and V2 and inversion of the T wave at the end of the surgical act, so it goes to intensive care unit, without anesthetic reversal and with orotracheal tube. Patient is monitored in ICU, EKG shows nodal rhythm, extubates at 12h post op and at 48h was transferred to cardiology where they establish that patient studies suggest Brugada pattern; Conclusions: With the increasing number of patients with inherited driving disorders who present the thee for non-cardiac surgery who are at risk of sudden death; success in peri, trans and postoperative management depends on a detailed knowledge of these conditions. (AU)

Radiofrequency treatment for electrical storm: evolution and monitoring. / Tratamiento con radiofrecuencia de la tormenta eléctrica: evolución y seguimiento.

Introducción y objetivos: La tormenta eléctrica (TE) se caracteriza por episodios repetidos de taquicardia ventricular o fibrilación ventricular relacionados con mal pronóstico a corto y largo plazos. El objetivo fue evaluar la prevalencia, resultados y supervivencia de los pacientes sometidos a tratamiento intervencionista por TE en un centro de referencia. Métodos: Estudio unicéntrico, observacional y retrospectivo. Se revisaron los procedimientos de ablación por TE y se evaluaron las características basales de los pacientes, tipo de procedimiento, mortalidad total, recurrencia de arritmia, mortalidad cardiovascular y necesidad de trasplante. Resultados: Desde enero de 2009 hasta diciembre de 2016 se realizaron 67 procedimientos (38% de complejos: 19% de ablación endoepicárdica, 7.5% de crioablación epicárdica quirúrgica, 3% de simpatectomía, 3% de inyección coronaria con alcohol; 6% de apoyo con oxigenación con membrana extracorpórea) en 41 pacientes (61% de causa isquémica) por TE. La mortalidad intraprocedimiento fue del 1.5%. La mediana de seguimiento fue de 23.5 meses (RIQ, 14.2-52.7). Tras el primer ingreso por TE (uno o varios procedimientos), la mortalidad a un año fue de 9.8%. La incidencia acumulada de trasplante cardiaco por TE fue de 2.4%. En el análisis multivariado, el riesgo de recurrencias arrítmicas o muerte por cualquier causa fue significativamente mayor en pacientes con arritmias clínicas inducibles (HR, 9.03; p = 0.017). Conclusiones: El tratamiento de pacientes con TE, instituido en un centro de referencia y con experiencia, se relacionó con una tasa baja de recurrencia y supervivencia elevada, con una tasa de trasplante cardiaco por TE muy baja. Ante una recurrencia temprana es recomendable practicar un nuevo procedimiento durante el ingreso. Introduction and objective: Electrical storm (ES) is characterized by repeated episodes of ventricular tachycardia or ventricular fibrillation, with poor short and long term prognosis. Our objective was to evaluate the prevalence, results of interventional treatment and survival of patients undergoing interventional treatment for ES in our center. Methods: Retrospective, unicentric and observational study. ES ablation procedures were revised and data regarding baseline characteristics of the patients, type of procedure, total mortality, recurrence of arrhythmia, cardiovascular mortality and the need for transplantation were evaluated. Results: From January 2009 to December 2016, 67 procedures (38% complex procedures: 19% epicardial ablation, 7.5% surgical epicardial crioablation, 3% simpatectomy, 3% coronary alcohol injection, 6% extracorporeal membrane oxygenation support) were performed in 41 patients (61% Ischemic etiology) due to ES. Intraprocedural mortality was 1.5%. The median follow-up was 23.5 months (IQR [14.2-52.7]). After the first admission for ES (one or several procedures), 1-year mortality was 9.8%. The cumulative incidence of cardiac transplantation was 2.4%. The risk of arrhythmic recurrences or death was significantly higher in patients with inducible clinical arrhythmias after ablation (HR: 9.03, p = 0.017). Conclusions: The treatment of patients with ES, performed in a reference center, allows obtaining good rates of recurrence and survival, with very low rates of cardiac transplantation for ES. In the presence of an early recurrence, it is advisable to perform a new procedure during admission.