Taquicardia ventricular polimórfica tipo Torsadess de Pointes secundaria a estimulación por marcapasos

La taquicardia ventricular polimórfica se origina en los ventrículos, cuyos complejos QRS son de morfología, amplitud y dirección variable, con frecuencias que oscilan entre 200 y 250 lpm, pudiendo ser autolimitadas o degenerar en una fibrilación ventricular. La TdP es un tipo de taquicardia ventricular polimórfica caracterizada por complejos con un eje eléctrico que gira alrededor de la línea isoeléctrica y que está asociada a QT largo. Se presenta el caso de una paciente portadora de marcapaso que presenta episodios de taquicardia ventricular polimórfica, con una morfología típica de TdP, sin documentación de QT prolongado previo ni actual, generada por la estimulación ventricular sobre onda T, de forma accidental por desplazamiento del electrodo auricular a Ventrículo Derecho (VD).

Polymorphic ventricular tachycardia is a tachycardia originating in the ventricles, where the QRS complexes have variable morphology, amplitude, and direction, with frequencies ranging between 200 and 250 bpm; it may be self-limited or degenerate into ventricular fibrillation. Torsades de Pointes (TdP) is a type of polymorphic ventricular tachycardia characterized by complexes with an electrical axis that rotates around the isoelectric line and that is associated with long QT interval. We present the case of a patient with a pacemaker who presents episodes of polymorphic ventricular tachycardia, with a typical morphology of TdP, without documentation of previous or current prolonged QT, generated by ventricular stimulation on the T wave, accidentally due to displacement of the atrial electrode to the Right Ventricle (RV).

Programmed ventricular stimulation in structural heart disease: Implications of patterns of ventricular arrhythmias induced to long-term outcomes

Introduction: Currently available data gives some credence to utility of VT induction studies in patients with stable ischemic cardiomyopathy, there are some unresolved questions as to define sensitive threshold for low-risk and the prognostic relevance of ill sustained or non-specific tachycardia on induction study. We evaluated potential ability of VT inducibility to predict likelihood of SHD (Structural heart disease) patients for subsequent arrhythmic or adverse cardiac events. Material and Methods: All consecutive patients with syncope/documented arrhythmia who had VT induction done were included and patients with VT storm, ACS,uncontrolled HF were excluded. We studied in 4 groups-monomorphic VT, sustained polymorphicVT, ill sustainedVT/VF and no VT/VF induced. The primary-endpoints were e Sudden death, all-cause mortality and secondary-endpoints were e MACE (AICD shock, death,HF, recurrence of VT). We screened 411 patients and included 169 within inducible (n ¼ 79) and non-inducible group (n ¼ 90). Results: There were a higher number of patients with coronary artery disease, LV dysfunction, patients on amiodarone in inducible group and no difference in usage of beta-blockers. Recurrence of VT, composite of MACE was significantly higher in inducible group (p < 0.05). Mortality was not different in 3 groups compared with no VT/VF group. We found that monomorphic VT group had significantly higher MACE as compared to others and also predicted recurrence of VT and AICD shock and showed a trend towards significance for prediction of mortality. Inducible patients on AICD had mortality similar to noninducible group. Conclusion: Induction of monomorphicVT/polymorphicVT with 3extrastimuli is associated with a higher number of MACE events on follow up. Induction of monomorphicVT predicts recurrence of VT/ICD shock.

A Young Healthy Woman with Supraventricular Tachycardia Reveals an Underlying Left Ventricular Non-compaction Cardiomyopathy: A Rare Case Report

LVNC (left ventricular non-compaction) is a rare congenital cardiomyopathy with a reported incidence of 0.05% in adults. It can occur in isolation or affect both ventricles. It’s characterized by prominent LV trabeculae and deep intertrabecular recesses which are filled with blood from the ventricular cavity without evidence of communication to the epicardial coronary artery system. Frequent premature supra ventricular tachycardia as unique finding in LVNC cardiomyopathy is rare manifestation of this disease. We report a case of a frequent persistent supraventricular tachycardia as first manifestation of a patient with LVNC cardiomyopathy in a young healthy woman who despite radio frequency ablation therapy of the supraventricular tachycardia remains symptomatic. The patient was later placed on medical therapy based on a non-cardio selective beta-blocker with a good clinical outcome without recurrent of supra-ventricular arrythmias.

Radiofrequency ablation for infants with incessant ventricular tachycardia and heart failure / 中国小儿急救医学

Objective:To investigate the effect of radiofrequency ablation on infants with incessant ventricular tachycardia(IIVT) and heart failure.Methods:Twenty-eight infants with IIVT combined with heart failure admitted to Beijing Children′s Hospital of Capital Medical University from January 2006 to December 2021 were selected, including 16 boys and 12 girls; 26 cases were treated with radiofrequency ablation.The characteristics of IIVT and heart failure and the results of radiofrequency ablation were analyzed.Results:The average age of the first diagnosis of 28 infants was 13.9 months old, and all of them had heart failure.Eleven infants had cardiogenic shock, three infants had cardiogenic syncope, two infants had respiratory failure for respiratory support, and one infant died.Color Doppler echocardiography showed that the left ventricular diameter increased and/or left ventricular systolic function decreased.Anti-heart failure treatment was effective in 27 cases(96.4%), electrical cardioversion in five cases, effective in three cases, and anti-arrhythmic drugs were effective in 17 cases(60.7%). Twenty-six cases(92.9%, 26/28) were treated with radiofrequency ablation, with immediate success in 23 cases(88.5%, 23/26) and effective in three cases(11.5%, 3/26). During the follow-up period for 3 to 36 months, cardiac function returned to normal in 25 cases(96.2%, 25/26) and recurred in three cases(11.5%, 3/26 cases), which were cured after radiofrequency ablation again.Temporary complications of atrioventricular block occurred in one case(3.8%, 1/26). In 26 cases of surgical children, 15 cases were measured by X-ray two-dimensional mapping and 11 cases were measured by three-dimensional mapping.The cumulative X-ray exposure was 87.0(51.5, 151.5) mGy and 1.2(0, 15.9) mGy, respectively, and the dose area product was 39.8(19.2, 427.8) μGy/m 2 and 2.8(0, 44.3) μGy/m 2.The cumulative X ray exposure and the dose area product were significantly reduced under the three-dimensional mapping method( P<0.001). Conclusion:The infants with IIVT combined with heart failure are prone to serious complications, and the effects of cardioversion and anti-arrhythmic drugs are limited.Radiofrequency ablation should be performed as soon as possible after heart failure is controlled.The application of three-dimensional mapping technology in surgery can significantly reduce ionizing radiation.

Ressonância Magnética Cardíaca como Ferramenta Diagnóstica Etiológica em Pacientes Recuperados de Morte Súbita Cardíaca ou Arritmias Ventriculares Instáveis / Cardiac Magnetic Resonance as an Etiological Diagnosis Tool in Recovered Sudden Cardiac Death or Unstable Ventricular Arrhythmia Patients

Resumo Fundamento A ressonância magnética cardíaca (RMC) tem relevância diagnóstica crescente em sobreviventes de morte súbita cardíaca (MSC) ou arritmia ventricular instável (AVI) em países desenvolvidos. Objetivo Procuramos avaliar retrospectivamente o papel adicional da RMC em um país em desenvolvimento com poucos recursos disponíveis e que pode direcionar um uso mais eficaz desses recursos. Métodos Foram incluídos sobreviventes de MSC ou AVI admitidos entre 2009 e 2019 em uma instituição acadêmica terciária após a realização de RMC. Dados demográficos, clínicos e laboratoriais foram coletados dos prontuários. Imagens e laudos de RMC foram analisados e o impacto disso no diagnóstico etiológico final foi afirmado. Realizou-se análise descritiva e definiu-se p<0,05 como significativo. Resultados Sessenta e quatro pacientes, 54,9±15,4 anos, sendo 42 (71,9%) do sexo masculino. A maioria dos eventos (81,3%) foi extra-hospitalar e a taquicardia ventricular foi o ritmo mais comum. Medicamentos cardiovasculares foram utilizados anteriormente por 55 pacientes, sendo os betabloqueadores os medicamentos mais utilizados (37,5%). O eletrocardiograma apresentava áreas elétricas inativas em 21,9% e todos apresentavam fibrose na RMC. A média da fração de ejeção do ventrículo esquerdo (FEVE) foi de 44±14%, com 60,9% ≤50% e apenas 29,7% ≤35%. Identificou-se realce tardio com gadolínio em 71,9%, com padrão transmural em 43,8%. A miocardiopatia chagásica foi a etiologia mais comum (28,1%), seguida da miocardiopatia isquêmica (17,2%). Entre 26 sem etiologia previamente identificada, foi possível definir com RMC (15 pacientes - 57%). Conclusão De acordo com estudos anteriores em países desenvolvidos, a RMC foi capaz de aumentar o diagnóstico etiológico e identificar o substrato arritmogênico, permitindo melhor atendimento em metade dos pacientes subdiagnosticados.

Abstract Background Cardiac magnetic resonance (CMR) has an increasing diagnostic relevance in survivors of sudden cardiac death (SCD) or unstable ventricular arrhythmia (UVA) in developed countries. Objective To evaluate retrospectively the additional role of CMR in a developing country where few resources are available, and should be used more effectively. Methods The study included SCD or UVA survivors admitted between 2009 and 2019 at a tertiary academic institution referred to CMR. Demographic, clinical, and laboratory data were collected from the medical records. CMR images and reports were reviewed and their impact on the final etiological diagnosis was determined. A descriptive analysis was performed and p<0.05 established as significant. Results Sixty-four patients, 54.9±15.4 years old, and 42 (71.9%) males. Most events (81.3%) were out of the hospital and ventricular tachycardia was the most common rhythm. Cardiovascular medications were previously used by 55 patients, and beta-blockers were the most used medications (37.5%). Electrocardiogram had electrical inactive areas in 21.9% and all of them had fibrosis at CMR. Mean left ventricular ejection fraction (LVEF) was 44±14%, with 60.9% ≤50% and only 29.7% ≤35%. Late gadolinium enhancement was identified in 71.9%, with a transmural pattern in 43.8%. Chagas cardiomyopathy was the most common etiology (28.1%), followed by ischemic cardiomyopathy (17.2%). Among 26 without a previously identified etiology, CMR could define it (15 patients - 57%). Conclusion In accordance with previous studies in developed countries, CMR was capable of increasing etiological diagnosis and identifying the arrhythmogenic substrate, allowing better care in half of the underdiagnosed patients.

Severe primary hypothyroidism presenting as polymorphic ventricular tachycardia / Hipotiroidismo primario grave que se presenta como taquicardia ventricular polimórfica

Abstract Introduction: Hypothyroidism may have various cardiovascular manifestations due to morphological, functional and electrical alterations in the heart. The usual electrocardiographic findings being sinus bradycardia, low voltage complexes, and slowed intraventricular conduction. Hypothyroidism manifesting as polymorphic ventricular tachycardia has only been reported in a few case reports. Clinical case. A 60-year-old lady presented to us in the emergency department in an unresponsive and unconscious state and electrocardiogram showed a polymorphic ventricular tachycardia. After initial resuscitation with direct current cardioversion and supportive care, she found to have severe hypothyroidism and responded well to thyroid replacement therapy. Conclusion. Polymorphic ventricular tachycardia is a life threatening emergency that can have various etiologies. Polymorphic ventricular tachycardia secondary to primary hypothyroidism is a rare presentation but it is treatable and reversible with thyroid replacement therapy. In patients presenting with QT interval prolongation and ventricular tachycardia, hypothyroidism should be one of the differential diagnosis.

Resumen Introducción: El hipotiroidismo puede presentar diferentes manifestaciones cardiovasculares dadas por alteraciones morfológicas, funcionales y eléctricas en el corazón, siendo los hallazgos electrocardiográficos usuales son la bradicardia sinusal, los complejos de bajo voltaje y la conducción intraventricular lenta. El hipotiroidismo manifestado como taquicardia ventricular polimórfica solo se ha descrito en unos pocos reportes de caso. Caso clínico: Se trata de una mujer de 60 años que acudió que acurdió al servicio de urgencias en un estado inconsciente y sin respuesta a estímulos, y el electrocardiograma reveló taquicardia ventricular polimórfica. Luego de la reanimación inicial con cardioversión con corriente directa y tratamiento sintomático se le encontró un hipotiroidismo grave, el cual se trató con terapia de reemplazo con hormona tiroidea. y se obtuvo una buena respuesta Conclusión. La taquicardia ventricular polimórfica es una emergencia vital que puede tener varias etiologías. La taquicardia ventricular polimórfica secundaria a un hipotiroidismo primario es una presentación poco común, pero es tratable y reversible con la terapia de reemplazo con hormona tiroidea. En los pacientes que presentan una prolongación del intervalo QT y taquicardia ventricular, es pertinente incluir el hipotiroidismo en el diagnóstico diferencial.

Taquicardia ventricular asociada a mutación en el gen de ankirina B / Ventricular tachycardia associated to a mutation in the ankyrin B gen

Resumen Se presenta el caso de una mujer de 14 años, con taquicardiomiopatía secundaria a taquicardia ventricular. Se evidenció la presencia de una variante de significado incierto en el gen ANK2, por lo que se consideró un posible síndrome de ankirina B. La paciente fue tratada con éxito a través de ablación con radiofrecuencia. Tras dicho procedimiento, tuvo recuperación completa de su función ventricular izquierda y resolución de los complejos ventriculares prematuros y los episodios de taquicardia ventricular.

Abstract We report a case of a 14-year-old with tachycardiomyopathy due to ventricular tachycardia. A variant of uncertain significance of the ANK2 gene was identified, which is suggestive of a possible ankyrin-B syndrome. The patient underwent a successful radiofrequency ablation. After the procedure, the patient completely recovered her left ventricular function and there was resolution of the premature ventricular complexes and ventricular tachycardia.

Ablación de taquicardia ventricular asistida con ECMO. Primera experiencia en nuestra institución / Ablation of ventricular tachycardia assisted with ECMO. First experience in our institution

Resumen La ablación con catéter de taquicardia ventricular ha demostrado mejorar la supervivencia y algunas veces es el único tratamiento efectivo, sobre todo en tormenta arrítmica; sin embargo la presencia de inestabilidad hemodinámica dificulta el procedimiento. El soporte con oxigenación por membrana extracorpórea (ECMO) es una alternativa para lograr el mapeo y ablación de taquicardia ventricular durante largos periodos de tiempo. Se presenta el caso de un paciente con cardiopatía isquémica y taquicardia ventricular con inestabilidad hemodinámica, en quien se realiza la ablación exitosa del sustrato mediante soporte con ECMO veno-arterial, sin episodios de taquicardia ventricular durante dos años de seguimiento.

Abstract Catheter ablation is useful for reducing drug refractory ventricular tachycardia (VT) episodes and can be life-saving when VT is incessant or arrhythmic storm. Left ventricular hemodynamic support may be required in patients with VT and hemodynamic instability. Extracorporeal membrane oxygenation (ECMO) support is an alternative to achieve ventricular tachycardia mapping and ablation over long periods of time. We present a case of successful catheter ablation of substrate in a patient with ischemic heart disease and ventricular tachycardia with hemodynamic instability performed using venous- arterial ECMO support. There were not episodes of ventricular tachycardia after 2 years of follow-up.

Una anomalía coronaria poco común como posible causa de taquicardia ventricular / A rare coronary anomaly as a possible cause of ventricular tachycardia

Resumen Se presenta el caso de un varón de 77 años que acude a urgencias tras un episodio sincopal en el contexto de una taquicardia ventricular monomorfa sostenida, por lo que se realizó cardioversión eléctrica. Para filiar la etiología del evento arrítmico ventricular se realizó un estudio de la anatomía coronaria mediante coronariografía invasiva, en el que se objetivaron las arterias coronarias sin lesiones significativas, pero como hallazgo casual se describió una anomalía coronaria, con ausencia de tronco coronario y salida independiente de las arterias circunfleja (Cx) y descendente anterior (DA) del seno coronario derecho, originándose la DA y la coronaria derecha del mismo ostium coronario. Dichos hallazgos se confirmaron mediante tomografía computarizada con reconstrucción tridimensional. Además, se objetivó un trayecto interarterial de la DA (entre las arterias aorta y pulmonar) y un trayecto intramuscular de la DA, así como un trayecto retroaórtico de la Cx. Debido a estos hallazgos, se procedió al implante de un desfibrilador automático implantable como prevención secundaria. El paciente tuvo una buena evolución posterior y fue dado de alta a su domicilio sin incidencias. Se presenta el caso para ayudar a comprender mejor estos trastornos, dado que actualmente constituyen un reto diagnóstico, ya que en muchas ocasiones se trata de un hallazgo casual en pruebas complementarias o incluso en autopsias. Además, es una causa relativamente frecuente de parada cardiorrespiratoria en pacientes jóvenes. De las muchas variables anatómicas que constituyen las anomalías coronarias, existe poca literatura sobre esta anomalía presentada y no hay imágenes similares a las de este caso.

Abstract We present the case of a 77-year-old man who came to the emergency room after a syncopal episode in the context of sustained monomorphic ventricular tachycardia for which electrical cardioversion was performed. In order to determine the etiology of the ventricular arrhythmic event, a study of the coronary anatomy was carried out using invasive coronary angiography, observing coronary arteries without significant lesions, although, as a chance finding, a coronary anomaly was described, with absence of the main coronary artery, with independent exit of circumflex (Cx) and anterior descending (AD) arteries of the right coronary sinus, originating the AD and right coronary artery from the same coronary ostium. These findings were later confirmed by computed tomography with 3D reconstruction. In addition, an interarterial path of AD (between aorta and pulmonary artery) and an intramuscular path of AD were observed, as well as a retro-aortic path of Cx. Given these findings, an implantable cardioverter defibrillator was implanted as secondary prevention. Good subsequent evolution with home discharge without incident. We present this case to help better understand these disorders, since they currently constitute a diagnostic challenge, since in many cases it is a chance finding in complementary tests or even in autopsies. It is also a relatively frequent cause of cardiorespiratory arrest in young patients. Of the many anatomical variables that make up the group of coronary anomalies, there is little bibliographic information on this anomaly presented, without finding images similar to those reported in this case.

Ventricular tachycardia and acute myocardial infarction induced by coronary artery spasm in patient without coronary artery disease

Coronary artery spasm, marked by coronary vasoconstriction, is one of the etiologies of myocardial ischemia, often presenting as vasospastic angina. Vasospastic angina is diagnosed when angina which predominantly occurs at rest, is accompanied by ST-segment changes in ECG, or in the setting of borderline ECG changes, a positive provocation test through coronary angiography is required. Although coronary artery spasms could manifest in wide clinical settings, the occurrence of ventricular arrhythmias and acute myocardial infarction solely caused by spasms without evidence of prior coronary artery disease is rare. This case report is about a 46-year-old man who presented with ventricular tachycardia and acute myocardial infarction that later was found to be secondary to coronary vasospasm observed directly through coronary angiography. We aim to emphasize the importance of coronary artery spasms as the etiology of malignant ventricular arrhythmias and acute myocardial infarction manifestation. Optimization in treatment and prevention shall reduce future life-threatening complications of coronary artery spasms.

Idiopathic left fascicular ventricular tachycardia in children and adolescents / Taquicardia ventricular fascicular izquierda idiopática en niños y adolescentes

Abstract Background: Idiopathic ventricular tachycardia (VT) in children with structurally normal hearts is generally unrelated to the risk of sudden arrhythmic death. Still, it may be associated with deterioration in the quality of life. VT involving the fascicular conduction system is the most typical form of idiopathic left VT. In this retrospective study, we describe the experience of the clinical presentation, catheter ablation, and long-term follow-up of left fascicular VT in children. Methods: An electrophysiological study was performed on consecutive children at a single tertiary center. Clinical fascicular left VT was induced by programmed stimulation, and catheter ablation was guided searching for Purkinje potentials. Results: We included 18 patients (0.8 patients/year): 14 (77.8%) males and four females. The mean age of the first VT episode was 8.5 ± 5 years. Intravenous verapamil administration was effective for paroxysmal fascicular VT but not for prevention of recurrences. The mean age at the time of catheter ablation was 11.1 ± 3.8 years (8 months-16 years). The mean weight was 36.8 ± 16.4 kg (8.7-58 kg). A 100% success rate was observed with catheter ablation after repeated procedures without major complications. Mean follow-up was 2.0 ± 1.2 years (1.0-4.0 years, median 1.5), with permanent success in all patients and no antiarrhythmic drug administration. Conclusions: Fascicular VT has an adverse clinical course in children. In most cases, this condition is drug refractory. Catheter ablation is successful and safe treatment and should represent the first-line approach in symptomatic children.

Resumen Introducción: La taquicardia ventricular (TV) idiopática en niños con corazón estructuralmente normal generalmente no se relaciona con el riesgo de muerte súbita arrítmica, pero puede asociarse con deterioro de la calidad de vida. La TV que involucra el sistema de conducción fascicular es la forma más común de TV izquierda idiopática. En este estudio retrospectivo se describe la experiencia de presentación clínica, ablación con catéter y seguimiento a largo plazo de TV fascicular en niños. Métodos: Se llevó a cabo un estudio electrofisiológico en niños consecutivos en un centro terciario. La TV fascicular clínica se indujo mediante la estimulación programada y la ablación con catéter fue guiada buscando el registro de potenciales de Purkinje. Resultados: Se incluyeron 18 pacientes (0.8 pacientes/año): 14 (77.8%) de sexo masculino y cuatro de sexo femenino. La media de edad a la cual ocurrió el primer episodio fue de 8.5 ± 5 años. La administración intravenosa de verapamilo fue eficaz para la TV fascicular paroxística, pero no para prevención de recurrencias. La media de edad de la ablación con catéter fue de 11.1 ± 3.8 años (8 meses-16 años). La media del peso fue 36.8 ± 16.4 kg (8.7-58 kg). Se observó el 100% de éxito con la ablación con catéter después de procedimientos repetidos sin complicaciones mayores. La media de seguimiento fue de 2.0 ± 1.2 años (1.0-4.0, mediana de 1.5 años) con éxito permanente en todos los pacientes y sin administración de fármacos antiarrítmicos. Conclusiones: En niños, el curso clínico de la TV fascicular es adverso. Además, en la mayoría de los casos, esta condición es refractaria a fármacos. La ablación con catéter resulta exitosa y segura y debe representar el abordaje de primera línea en niños sintomáticos.

Arritmias ventriculares en el síndrome de takotsubo: cuestión de sexo / Sex-related risk of arrhythmia in takotsubo syndrome

Resumen El síndrome de takotsubo, también conocido como miocardiopatía de estrés, representa un difícil reto diagnóstico, pues en muchos casos su presentación es superponible al infarto de miocardio por ruptura de placa; el diagnóstico definitivo se basa en la ausencia de lesiones culpables en la coronariografía. La fisiopatología de la enfermedad no está por completo establecida, y tiene un pronóstico generalmente benigno. Sin embargo, existe un porcentaje no despreciable de pacientes que sufren complicaciones graves, entre las que destacan las arritmias malignas tipo taquicardia ventricular polimórfica por prolongación del intervalo QT. A pesar de que el síndrome de takotsubo afecta principalmente a las mujeres, quienes por otra parte también suelen tener intervalos QT más prolongados en condiciones basales, la muerte súbita de origen arrítmico aparece con mayor frecuencia en los hombres que sufren esta enfermedad. Se presentan dos casos de ensanchamiento extremo del intervalo QT corregido en pacientes con takotsubo que tuvieron desenlaces diferentes. El propósito de este trabajo es destacar y revisar las diferencias electrocardiográficas y pronósticas relacionadas con el sexo de los sujetos que desarrollan esta controvertida enfermedad.

Abstract Takotsubo syndrome, also known as stress cardiomyopathy, is a difficult diagnostic challenge as, in many cases, its presentation can overlap with that of myocardial infarction due to plaque rupture. The definitive diagnosis is based on the lack of culprit lesions on coronariography. The pathophysiology of the disease has not been completely ascertained, and it has a generally benign prognosis. However, a not inconsiderable percentage of patients experience serious complications, notably malignant arrhythmias like polymorphic ventricular tachycardia due to a prolonged QT interval. Despite takotsubo syndrome affecting mainly women who, furthermore, generally have longer basal QT intervals, sudden death due to arrhythmias is more common in men with this disease. Two cases are presented of extremely prolonged corrected QT intervals in patients with takotsubo, with different outcomes. The purpose of this paper is to highlight and review the electrocardiographic and prognostic differences related to the gender of the individuals who develop this controversial disease.

Ablación y neuromodulación en tormenta arrítmica: serie de 4 casos y revisión de la literatura / Ablation and neuromodulation in arrhythmic storm: a report of 4 cases and literature review

Resumen: Se presenta una serie de 4 casos clínicos de pacientes con y sin cardiopatía estructural, que tuvieron uno o más episodios de tormenta arrítmica. Se describen los tratamientos con sus resultados y una revisión bibliográfica con los avances en el tema más allá de la ablación con catéter.

Abstract: We present 4 clinical cases of patients with and without structural heart disease, who had one or more episodes of arrhythmic storm. Treatments, results and a bibliographic review with advances beyond catheter ablation are described.

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.

Bidirectional Ventricular Tachycardia Due to Digitalis Intoxication

Bidirectional ventricular tachycardia (BDVT) is defined by beat-to-beat alternation of the QRS axis on the electrocardiogram. Its diagnosis is uncommon, and the most characteristic etiology is digitalis intoxication (DI). We report the case of a patient with heart failure of valve origin admitted for sepsis that progressed to BDVT and death, associated with DI.

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.

Peculiaridade dos pacientes com arritmias hereditárias na pandemia pela COVID-19 / Peculiar aspects of patients with Inherited arrhythmias during the COVID-19 pandemic

Resumo Desde dezembro de 2019, observamos o rápido avanço da síndrome respiratória aguda grave causada pelo coronavírus 2019 (SARS-CoV-2). O impacto da evolução clínica de uma infecção respiratória é pouco conhecido em pacientes portadores de arritmias hereditárias, devido à baixa prevalência dessas doenças. Os pacientes que apresentam quadros infecciosos podem exacerbar arritmias primárias ocultas ou bem controladas, por diversos fatores, tais como febre, distúrbios eletrolíticos, interações medicamentosas, estresse adrenérgico e, eventualmente, o próprio dano miocárdico do paciente séptico. O objetivo desta revisão é destacar os principais desafios que podemos encontrar durante a pandemia pela Covid 19, especificamente nos pacientes com arritmias hereditárias, com destaque para a síndrome do QT longo congênito (SQTL), a síndrome de Brugada (SBr), a taquicardia ventricular polimórfica catecolaminérgica (TVPC) e a cardiomiopatia arritmogênica do ventrículo direito.

Abstract Since December 2019 we have observed the rapid advance of the severe acute respiratory syndrome caused by the new coronavirus (SARS-CoV-2). The impact of the clinical course of a respiratory infection is little known in patients with hereditary arrhythmias, due to the low prevalence of these diseases. Patients who present with infectious conditions may exacerbate hidden or well-controlled primary arrhythmias, due to several factors, such as fever, electrolyte disturbances, drug interactions, adrenergic stress and, eventually, the septic patient's own myocardial damage. The aim of this review is to highlight the main challenges we may encounter during the Covid 19 pandemic, specifically in patients with hereditary arrhythmias, with emphasis on the congenital long QT syndrome (LQTS), Brugada syndrome (SBr), ventricular tachycardia polymorphic catecholaminergic (CPVT) and arrhythmogenic right ventricular cardiomyopathy.