Searching for genetic modulators of the phenotypic heterogeneity in Brugada syndrome.

In Brugada syndrome, even within the same family where all affected individuals share the same mutation, phenotypic variation is prominent, with variable penetrance and expressivity, presenting different degrees of involvement. It is difficult to establish a direct correlation between genotype and phenotype to predict prognosis in complications and risk of sudden death. The factors that modulate this inter- and intra-familial phenotypic variability remain to be determined. With the intention of testing whether other genetic factors, in addition to the causal mutation in SCN5A, may have a modulating effect on the Brugada phenotype and the risk of sudden death, we have studied 8 families with a causal variant in SCN5A with at least two affected individuals, one of whom has suffered cardiac arrest or sudden death. Whole exome sequencing was performed looking for additional variants that modify the phenotype and allow us to predict a better or worse prognosis for the evolution of the disease. The results did not show any clear genetic modifier; nevertheless, highlight the possible implication of the cholesterol and fibrosis pathways, as well as the circadian rhythm, as possible modulators of Brugada syndrome phenotype.

Frecuentación y motivos de asistencia en el servicio de urgencias tras la ablación de fibrilación auricular con criobalón / Emergency department attendance and reasons for consultation after cryoballoon ablation for pulmonary vein isolation of atrial fibrillation

Introducción y objetivos Existe poca información sobre la frecuentación a urgencias en las semanas siguientes a un procedimiento de aislamiento de venas pulmonares con criobalón. El objetivo del estudio es cuantificar las visitas a urgencias en los primeros 3 meses tras el procedimiento, evaluando los motivos, el diagnóstico final y la actitud terapéutica. Métodos Estudio observacional retrospectivo sobre 330 pacientes sometidos a un primer procedimiento de crioablación de fibrilación auricular. Se realizó un seguimiento de 90 días tras el procedimiento durante el que se registraron las visitas a urgencias con los síntomas que motivaron la consulta, electrocardiograma, actitud terapéutica y diagnóstico final, clasificado como alteración del ritmo, complicación confirmada, complicación posible y sin relación con el procedimiento ni con el trastorno arrítmico. Resultados Un total de 112 pacientes (34%) consultaron en urgencias, 50 de ellos (44,6%) por palpitaciones. En 44 pacientes (39,3%) se documentó una arritmia auricular sostenida. De las 29 consultas (25,9%) por síntomas potencialmente relacionados con complicaciones del procedimiento, 5 fueron complicaciones confirmadas de la punción inguinal, 10 quedaron con diagnóstico no confirmado de posible complicación y 41 visitas no guardaron relación con el procedimiento. El 21,4% del total de consultas fueron por palpitaciones en las que no se tomó ninguna medida terapéutica. Conclusiones Un tercio de los pacientes consultaron en urgencias al menos 1 vez, y los síntomas arrítmicos fueron el motivo más frecuente. Las complicaciones diferidas del procedimiento son raras y, en general, benignas. La disponibilidad de un sistema de teleconsulta con transmisión del ECG a distancia podría evitar hasta un 20% de los desplazamientos a urgencias. (AU)

Introduction and objectives There are few data on emergency visits after cryoballoon-based pulmonary vein isolation. The aim of this study was to quantify emergency department visits during the first 3 months after the procedure and to identify the reasons for consultation, final diagnoses, and the therapeutic approach. Methods Observational, retrospective study of 330 consecutive patients undergoing a first cryoballoon-based ablation procedure. Patients were followed up for 90 days after the procedure. We recorded emergency visits, symptoms, electrocardiographic data, and the therapeutic approach. Final diagnoses were classified as rhythm disorder, confirmed complication, possible complication, and unrelated to the procedure or to the arrhythmic disorder. Results A total of 112 (34%) patients attended the emergency department, 50 (44.6%) for palpitations. Sustained atrial arrhythmias were documented in 44 (39.3%) patients. Among the 29 (25.9%) visits for complications potentially related to the procedure, 5 were confirmed inguinal puncture complications and 10 were classified as unconfirmed possible complications. Forty-one visits were unrelated to the procedure or to the arrhythmic disorder. A total of 21.4% of the visits were due to palpitations requiring no therapeutic action. Conclusions A third of the patients attended the emergency department at least once, with the most frequent reason being arrhythmia-related symptoms. Late complications were rare and generally mild. Up to 20% of emergency visits could potentially be avoided by the availability of a teleconsulting system with remote electrocardiogram transmission. (AU)

Emergency department attendance and reasons for consultation after cryoballoon ablation for pulmonary vein isolation of atrial fibrillation.

INTRODUCTION AND OBJECTIVES: There are few data on emergency visits after cryoballoon-based pulmonary vein isolation. The aim of this study was to quantify emergency department visits during the first 3 months after the procedure and to identify the reasons for consultation, final diagnoses, and the therapeutic approach. METHODS: Observational, retrospective study of 330 consecutive patients undergoing a first cryoballoon-based ablation procedure. Patients were followed up for 90 days after the procedure. We recorded emergency visits, symptoms, electrocardiographic data, and the therapeutic approach. Final diagnoses were classified as rhythm disorder, confirmed complication, possible complication, and unrelated to the procedure or to the arrhythmic disorder. RESULTS: A total of 112 (34%) patients attended the emergency department, 50 (44.6%) for palpitations. Sustained atrial arrhythmias were documented in 44 (39.3%) patients. Among the 29 (25.9%) visits for complications potentially related to the procedure, 5 were confirmed inguinal puncture complications and 10 were classified as unconfirmed possible complications. Forty-one visits were unrelated to the procedure or to the arrhythmic disorder. A total of 21.4% of the visits were due to palpitations requiring no therapeutic action. CONCLUSIONS: A third of the patients attended the emergency department at least once, with the most frequent reason being arrhythmia-related symptoms. Late complications were rare and generally mild. Up to 20% of emergency visits could potentially be avoided by the availability of a teleconsulting system with remote electrocardiogram transmission.

Medium- and long-term outcome after a single second-generation cryoballoon ablation in patients with paroxysmal and persistent atrial fibrillation.

INTRODUCTION AND OBJECTIVES: To report medium- and long-term results following a single second-generation cryoballoon (CB2)-based ablation procedure in patients with paroxysmal (PAF) and persistent (PeAF) atrial fibrillation. METHODS: A retrospective study was performed of consecutive patients undergoing a first CB2-based ablation procedure in a tertiary center. Cryoenergy was applied for 3 min if a time to effect <60 s was documented or 4 min otherwise, with a bonus application in cases of late isolation or suboptimal temperature. Follow-up was obtained from the regional health electronic records system and by telephone or personal interviews. Recurrence was defined as any atrial arrhythmia >30 s beyond a three-month blanking period. The clinical impact of recurrences was classified using a severity score. RESULTS: A total of 172 patients (134 PAF and 38 PeAF) were included, of whom 25 (14.5%) had structural heart disease and 120 (69.7%) had a normal or mildly dilated left atrium. Acute success was achieved in 167 (97.1%). After a median follow-up of 27 (14-41) months, 100 patients (58.1%) remained free of atrial arrhythmias (64.2% for PAF and 36.8% for PeAF, p=0.006). Left atrial size (p=0.05) and clinical presentation as PeAF (p=0.006) were predictors of recurrence. Of patients with recurrences, 11.1% did not require further therapies and an additional 16.7% had good control with antiarrhythmic drugs. CONCLUSIONS: A single CB2 procedure resulted in 58.1% of patients remaining free of atrial arrhythmias at 27-month follow-up. Conservative management was useful in 27.8% of patients with recurrences.

Valor del «test de bipedestación» en el diagnóstico y la evaluación de la respuesta al tratamiento con bloqueadores beta en el síndrome de QT largo / Value of the 'standing test' in the diagnosis and evaluation of beta-blocker therapy response in long QT syndrome

Introducción y objetivos: Los pacientes con síndrome de QT largo (SQTL) tienen una adaptación anormal del QT a los cambios bruscos de la frecuencia cardiaca producidos con la bipedestación. Este trabajo estudia la utilidad del test de bipedestación en una cohorte de pacientes con SQTL y evalúa si el fenómeno de «mala adaptación» del QT se normaliza con el tratamiento con bloqueadores beta. Métodos: Se realizó un electrocardiograma basal y otro inmediatamente tras la bipedestación a 36 pacientes con SQTL (6 [17%] con QTL1, 20 [56%] con QTL2, 3 [8%] con QTL7 y 7 [19%] con genotipo no identificado) y 41 controles. Se midió el intervalo QT corregido (QTc) basal (QTcdecúbito) y tras la bipedestación (QTcbipedestación) y el incremento del QTc (ΔQTc = QTcbipedestación - QTcdecúbito). Se repitió el test en 26 de los pacientes bajo tratamiento con bloqueadores beta. Resultados: El QTcbipedestación y el ΔQTc fueron mayores en el grupo de SQTL que en el grupo control (QTcbipedestación, 528 ± 46 frente a 420 ± 15 ms; p < 0,0001; ΔQTc, 78 ± 40 frente a 8 ± 13 ms; p < 0,0001). No hubo diferencias significativas entre los pacientes con QTL1 y QTL2. Los pacientes con SQTL presentaron patrones típicos del segmento ST-onda T tras la bipedestación. Las curvas receiver operating characteristic del QTcbipedestación y ΔQTc mostraron un incremento significativo del valor diagnóstico comparadas con la del QTcdecúbito(área bajo la curva de ambos, 0,99 frente a 0,85; p < 0,001). El tratamiento con bloqueadores beta atenuó la respuesta a la bipedestación de los pacientes con SQTL (en tratamiento, QTcbipedestación, 440 ± 32 ms [p < 0,0001] y ΔQTc, 14 ± 16 ms [p < 0,0001]). Conclusiones: La evaluación del intervalo QTc tras la bipedestación proporciona un alto rendimiento diagnóstico y podría ser de gran utilidad en la monitorización del tratamiento con bloqueadores beta en los pacientes con SQTL (AU)

Introduction and objectives: Patients with congenital long QT syndrome (LQTS) have an abnormal QT adaptation to sudden changes in heart rate provoked by standing. The present study sought to evaluate the standing test in a cohort of LQTS patients and to assess if this QT maladaptation phenomenon is ameliorated by beta-blocker therapy. Methods: Electrographic assessments were performed at baseline and immediately after standing in 36 LQTS patients (6 LQT1 [17%], 20 LQT2 [56%], 3 LQT7 [8%], 7 unidentified-genotype patients [19%]) and 41 controls. The corrected QT interval (QTc) was measured at baseline (QTcsupine) and immediately after standing (QTcstanding); the QTc change from baseline (ΔQTc) was calculated as QTcstanding - QTcsupine. The test was repeated in 26 patients receiving beta-blocker therapy. Results: Both QTcstanding and ΔQTc were significantly higher in the LQTS group than in controls (QTcstanding, 528 ± 46 ms vs 420 ± 15 ms, P < .0001; ΔQTc, 78 ± 40 ms vs 8 ± 13 ms, P < .0001). No significant differences were noted between LQT1 and LQT2 patients. Typical ST-T wave patterns appeared after standing in LQTS patients. Receiver operating characteristic curves of QTcstanding and ΔQTc showed a significant increase in diagnostic value compared with the QTcsupine (area under the curve for both, 0.99 vs 0.85; P < .001). Beta-blockers attenuated the response to standing in LQTS patients (QTcstanding, 440 ± 32 ms, P < .0001; ΔQTc, 14 ± 16 ms, P < .0001). Conclusions: Evaluation of the QTc after the simple maneuver of standing shows a high diagnostic performance and could be important for monitoring the effects of beta-blocker therapy in LQTS patients (AU)

Value of the "Standing Test" in the Diagnosis and Evaluation of Beta-blocker Therapy Response in Long QT Syndrome.

INTRODUCTION AND OBJECTIVES: Patients with congenital long QT syndrome (LQTS) have an abnormal QT adaptation to sudden changes in heart rate provoked by standing. The present study sought to evaluate the standing test in a cohort of LQTS patients and to assess if this QT maladaptation phenomenon is ameliorated by beta-blocker therapy. METHODS: Electrographic assessments were performed at baseline and immediately after standing in 36 LQTS patients (6 LQT1 [17%], 20 LQT2 [56%], 3 LQT7 [8%], 7 unidentified-genotype patients [19%]) and 41 controls. The corrected QT interval (QTc) was measured at baseline (QTcsupine) and immediately after standing (QTcstanding); the QTc change from baseline (ΔQTc) was calculated as QTcstanding - QTcsupine. The test was repeated in 26 patients receiving beta-blocker therapy. RESULTS: Both QTcstanding and ΔQTc were significantly higher in the LQTS group than in controls (QTcstanding, 528 ± 46ms vs 420 ± 15ms, P < .0001; ΔQTc, 78 ± 40ms vs 8 ± 13ms, P < .0001). No significant differences were noted between LQT1 and LQT2 patients. Typical ST-T wave patterns appeared after standing in LQTS patients. Receiver operating characteristic curves of QTcstanding and ΔQTc showed a significant increase in diagnostic value compared with the QTcsupine (area under the curve for both, 0.99 vs 0.85; P < .001). Beta-blockers attenuated the response to standing in LQTS patients (QTcstanding, 440 ± 32ms, P < .0001; ΔQTc, 14 ± 16ms, P < .0001). CONCLUSIONS: Evaluation of the QTc after the simple maneuver of standing shows a high diagnostic performance and could be important for monitoring the effects of beta-blocker therapy in LQTS patients.

Fenotipo heterogéneo del síndrome de QT largo causado por la mutación KCNH2-H562R: importancia del estudio genético familiar / Heterogeneous phenotype of long QT Syndrome caused by the KCNH2-H562R Mutation: Importance of familial genetic testing

Introducción y objetivos: El síndrome de QT largo es una canalopatía hereditaria que se asocia a síncope y muerte súbita. La heterogeneidad fenotípica de esta enfermedad hace que el estudio genético sea fundamental para detectar a los sujetos con síndrome de QT largo oculto. En este trabajo se exponen las características de una familia con 13 portadores de la mutación missense KCNH2-H562R que afecta a la región del poro del canal HERG. Métodos: Se describió la mutación KCNH2-H562R en un varón de 65 años con intervalo QTc prolongado que presentó un episodio de torsade de pointes. Posteriormente, se identificaron 13 portadores de la mutación en la familia. Se realizó evaluación clínica, electrocardiograma y ecocardiograma a los portadores (edad, 48 ± 26 años; el 46% varones). Resultados: El QTc medio en los portadores fue de 493 ± 42 ms (3 [23%] mostraron QTc normal); 6 (46%) tuvieron síntomas (4, síncope; 1, muerte súbita; 1, muerte súbita resucitada [probando]). Durante el tratamiento con bloqueadores beta, 11 (92%) de los 12 portadores permanecieron asintomáticos a los 5 años de seguimiento (1 paciente requirió simpatectomía cardiaca izquierda). El acortamiento del QTc con bloqueadores beta fue de 50 ± 37 ms. Hubo 1 muerte súbita en un paciente que rechazó tratamiento con bloqueadores beta. Conclusiones: El estudio familiar es fundamental en la interpretación de los resultados de los tests genéticos en la actualidad. Este artículo describe el fenotipo variable y heterogéneo de una amplia familia portadora de la mutación KCNH2-H562R y destaca el papel del estudio genético en la identificación de los individuos en riesgo que se beneficiarían del tratamiento con bloqueadores beta (AU)

Introduction and objectives: Long QT syndrome is an inherited ion channelopathy that leads to syncope and sudden death. Because of the heterogeneous phenotype of this disease, genetic testing is fundamental to detect individuals with concealed long QT syndrome. In this study, we determined the features of a family with 13 carriers of the KCNH2-H562R missense mutation, which affects the pore region of the HERG channel. Methods: We identified the KCNH2-H562R mutation in a 65-year-old man with a prolonged QTc interval who had experienced an episode of torsade de pointes. Subsequently, a total of 13 mutation carriers were identified in the family. Carriers (age 48 [26] years; 46% males) underwent clinical evaluation, electrocardiography and echocardiography. Results: The mean (standard deviation) QTc in carriers was 493 (42) ms (3 [23%] showed normal QTc); 6 (46%) had symptoms (4, syncope; 1, sudden death; 1, aborted sudden death [proband]). While under treatment with beta-blockers, 11 of 12 carriers (92%) remained asymptomatic at 5 years of follow-up (1 patient required left cardiac sympathectomy). The QTc shortening with beta-blockers was 50 (37) ms. There was 1 sudden death in a patient who refused treatment. Conclusions: Family study is essential in the interpretation of a genetic testing result. This article describes the heterogeneous and variable phenotype of a large family with the KCNH2-H562R mutation and highlights the role of genetic study for the appropriate identification of at-risk individuals who would benefit from treatment (AU)

Mutación p. Arg14del en fosfolambán en una familia española con miocardiopatía arritmogénica: evidencia de una mutación europea fundadora / Phospholamban p.arg14del mutation in a spanish family with arrhythmogenic cardiomyopathy: evidence for a european founder mutation

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Heterogeneous Phenotype of Long QT Syndrome Caused by the KCNH2-H562R Mutation: Importance of Familial Genetic Testing.

INTRODUCTION AND OBJECTIVES: Long QT syndrome is an inherited ion channelopathy that leads to syncope and sudden death. Because of the heterogeneous phenotype of this disease, genetic testing is fundamental to detect individuals with concealed long QT syndrome. In this study, we determined the features of a family with 13 carriers of the KCNH2-H562R missense mutation, which affects the pore region of the HERG channel. METHODS: We identified the KCNH2-H562R mutation in a 65-year-old man with a prolonged QTc interval who had experienced an episode of torsade de pointes. Subsequently, a total of 13 mutation carriers were identified in the family. Carriers (age 48 [26] years; 46% males) underwent clinical evaluation, electrocardiography and echocardiography. RESULTS: The mean (standard deviation) QTc in carriers was 493 (42) ms (3 [23%] showed normal QTc); 6 (46%) had symptoms (4, syncope; 1, sudden death; 1, aborted sudden death [proband]). While under treatment with beta-blockers, 11 of 12 carriers (92%) remained asymptomatic at 5 years of follow-up (1 patient required left cardiac sympathectomy). The QTc shortening with beta-blockers was 50 (37) ms. There was 1 sudden death in a patient who refused treatment. CONCLUSIONS: Family study is essential in the interpretation of a genetic testing result. This article describes the heterogeneous and variable phenotype of a large family with the KCNH2-H562R mutation and highlights the role of genetic study for the appropriate identification of at-risk individuals who would benefit from treatment.

Utilidad de un catéter de ablación con sensor de fuerza en la evaluación de las áreas de cicatriz miocárdicas de pacientes con taquicardia ventricular / Usefulness of the contact force sensing catheter to assess the areas of myocardial scar in patients with ventricular tachycardia

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Respuesta al ECG de diciembre de 2014 / Response to ECG, December 2014

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Narrow irregular QRS tachycardia with AV dissociation: what is the mechanism?

A 39-year-old woman with no structural heart disease and frequent episodes of sudden onset palpitations was referred for the electrophysiological study. During the study, a slightly irregular narrow QRS tachycardia with AV dissociation was repeatedly induced and spontaneously terminated. Apparently, irregular cycles and termination of the tachycardia were related to the dissociated sinus rhythm: atrial depolarizations timed when the AV junction was refractory were able to reset the tachycardia, while early atrial depolarizations caused its termination. This observation was enough to diagnose the tachycardia mechanism in our case.

Multiple interatrial electrical connection after heart transplantation.

A 62-year-old woman who underwent heart transplantation 6 years later presented a regular atrial tachycardia. Electrophysiologic evaluation showed an atrial arrhythmia in the recipient atrium with 2:1 conduction to the donor atrium, with a confusing electroanatomical map. With the suspect of alternant conduction through two different breakthroughs, the map was split in two concordant maps, corresponding to two connections that were successfully ablated. Later on, a third connection was detected and therefore ablated.

Identificación del nervio frénico con tomografía computarizada cardiaca multidetector / Phrenic nerve identification with cardiac multidetector computed tomography

La crioablación con balón de las venas pulmonares es una técnica novedosa que se ha demostrado útil para prevenir las recurrencias de la fibrilación auricular paroxística y persistente. Una de las complicaciones más relevantes del procedimiento es la parálisis del nervio frénico derecho. Se ha descrito recientemente la utilidad de la tomografía computarizada multidetector para localizar el recorrido de la arteria y el nervio frénico y predecir el riesgo de parálisis frénica durante la crioablación, según la distancia del paquete vasculonervioso frénico derecho al ostium de la vena pulmonar superior derecha. A 55 pacientes consecutivos con fibrilación auricular paroxística (media de edad 52±12 años) sometidos a crioablación con balón, se les realizó estudio previo con tomografía computarizada multidetector para medir el tamaño de las venas pulmonares. Se pudo identificar algún fragmento de la arteria pericardiofrénica derecha en 10 pacientes (20%), con una longitud media de 25 (7-68) mm (AU)

Cryoballoon ablation of the pulmonary veins is a new technique that has proven useful in preventing paroxysmal and persistent atrial fibrillation recurrence. One of the most serious complications of this method is right phrenic nerve palsy. The usefulness of multidetector computed tomography to locate the right phrenic nerve and artery and predict the risk of phrenic nerve palsy during cryoablation according to the distance between the right phrenic neurovascular bundle and the right superior pulmonary vein ostium has recently been described. Fifty-five consecutive patients with paroxysmal atrial fibrillation (52±12 years) underwent balloon cryoablation, following multidetector computed tomography to measure the pulmonary veins. We were able to identify segments of the right pericardiacophrenic artery (mean length 25mm [range 7-68mm]) in only 10 patients (20%) (AU)

[Phrenic nerve identification with cardiac multidetector computed tomography]. / Identificación del nervio frénico con tomografía computarizada cardiaca multidetector.

Cryoballoon ablation of the pulmonary veins is a new technique that has proven useful in preventing paroxysmal and persistent atrial fibrillation recurrence. One of the most serious complications of this method is right phrenic nerve palsy. The usefulness of multidetector computed tomography to locate the right phrenic nerve and artery and predict the risk of phrenic nerve palsy during cryoablation according to the distance between the right phrenic neurovascular bundle and the right superior pulmonary vein ostium has recently been described. Fifty-five consecutive patients with paroxysmal atrial fibrillation (52 ± 12 years) underwent balloon cryoablation, following multidetector computed tomography to measure the pulmonary veins. We were able to identify segments of the right pericardiacophrenic artery (mean length 25 mm [range 7-68 mm]) in only 10 patients (20%).

Premature ventricular complexes as a trigger for ventricular fibrillation.

INTRODUCTION AND OBJECTIVES: The mechanisms that trigger ventricular fibrillation (VF) are poorly understood. The aim of this study was to analyze the initiation of VF in electrograms stored in implantable cardioverter-defibrillators (ICDs). METHODS: We analyzed ICD electrograms from patients who had suffered at least one episode of VF. RESULTS: Of 250 patients with ICDs, 13 (10 male and 3 female, age 49+/-22 years) had at least one episode of VF. The diagnoses were Brugada syndrome (n=4), ischemic heart disease (n=3), dilated cardiomyopathy (n=2), hypertrophic cardiomyopathy (n=1), short-coupled variant of torsades de pointes (n=1), endocardial fibroelastosis (n=1) and idiopathic VF (n=1). In 7 patients, VF was the reason for ICD implantation. Overall, 31 episodes of VF were recorded, including three episodes of arrhythmic storm. In the 7 patients who had more than one episode of VF (within minutes or up to 3 years apart), all episodes started with premature ventricular complexes (PVCs) that had the same morphology and similar coupling intervals. A short-long-short cycle was observed in 2 patients. In 21 episodes, PVCs that did not trigger VF were observed during sinus rhythm. There was no significant difference between them and PVCs that did trigger VF in terms of morphology, coupling interval (409+/-121 ms vs. 411+/-123 ms) or the preceding sinus rhythm RR interval (801+/-233 ms vs. 793+/-230 ms). CONCLUSIONS: Spontaneous VF in the form of an arrhythmic storm or an isolated episode were triggered by PVCs. On occasions, PVCs preceded VF without triggering it.

Extrasistolia ventricular desencadenante de la fibrilación ventricular / Premature ventricular complexes as a trigger for ventricular fibrillation

Introducción y objetivos. Los mecanismos de inicio de la fibrilación ventricular (FV) son poco conocidos. El objetivo de este estudio es analizar el inicio de la FV en los electrogramas almacenados en los desfibriladores automáticos implantables (DAI). Métodos. Hemos analizado los electrogramas de pacientes con DAI y al menos un episodio de FV. Resultados. De una población de 250 pacientes portadores de DAI, 13 tuvieron al menos un episodio de FV, 10 varones y 3 mujeres (edad, 49 ± 22 años), diagnosticados de síndrome de Brugada (n = 4), cardiopatía isquémica (n = 3), miocardiopatía dilatada (n = 2), miocardiopatía hipertrófica (n = 1), torsades de pointes por extrasístole ventricular (EV) con acoplamiento corto (n = 1), fibroelastosis cardiaca (n = 1) y FV idiopática (n = 1). En 7 pacientes la FV fue el motivo del implante. Se registraron 31 episodios de FV (3 tormentas arrítmicas). En cada paciente, todos los episodios comenzaron con una EV de la misma morfología y similar intervalo de acoplamiento en los 7 pacientes con más de un episodio (minutos-3 años). Se objetivó ciclo corto-largo-corto en 2 pacientes. En 21 episodios, se registraron EV en ritmo sinusal que no desencadenaron FV. La morfología, el intervalo de acoplamiento (409 ± 121 frente a 411 ± 123 ms) y el ciclo del latido sinusal precedente (801 ± 233 frente a 793 ± 230 ms) no presentaron diferencias significativas al compararlas con las EV inductoras de FV. Conclusiones. La FV espontánea se desencadena por EV en las tormentas arrítmicas y en episodios aislados. En ocasiones las EV preceden a la FV sin desencadenarla (AU)

Introduction and objectives. The mechanisms that trigger ventricular fibrillation (VF) are poorly understood. The aim of this study was to analyze the initiation of VF in electrograms stored in implantable cardioverterdefibrillators (ICDs). Methods. We analyzed ICD electrograms from patients who had suffered at least one episode of VF. Results. Of 250 patients with ICDs, 13 (10 male and 3 female, age 49±22 years) had at least one episode of VF. The diagnoses were Brugada syndrome (n=4), ischemic heart disease (n=3), dilated cardiomyopathy (n=2), hypertrophic cardiomyopathy (n=1), short-coupled variant of torsades de pointes (n=1), endocardial fibroelastosis (n=1) and idiopathic VF (n=1). In 7 patients, VF was the reason for ICD implantation. Overall, 31 episodes of VF were recorded, including three episodes of arrhythmic storm. In the 7 patients who had more than one episode of VF (within minutes or up to 3 years apart), all episodes started with premature ventricular complexes (PVCs) that had the same morphology and similar coupling intervals. A short-long-short cycle was observed in 2 patients. In 21 episodes, PVCs that did not trigger VF were observed during sinus rhythm. There was no significant difference between them and PVCs that did trigger VF in terms of morphology, coupling interval (409±121 ms vs. 411±123 ms) or the preceding sinus rhythm RR interval (801±233 ms vs. 793±230 ms). Conclusions. Spontaneous VF in the form of an arrhythmic storm or an isolated episode were triggered by PVCs. On occasions, PVCs preceded VF without triggering it (AU)