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1.
J Cardiovasc Electrophysiol ; 35(5): 906-915, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38433355

RESUMEN

INTRODUCTION: Right ventricular (RV) pacing sometimes causes left ventricular (LV) systolic dysfunction, also known as pacing-induced cardiomyopathy (PICM). However, the association between specifically paced QRS morphology and PICM development has not been elucidated. This study aimed to investigate the association between paced QRS mimicking a complete left bundle branch block (CLBBB) and PICM development. METHODS: We retrospectively screened 2009 patients who underwent pacemaker implantation from 2010 to 2020 in seven institutions. Patients who received pacemakers for an advanced atrioventricular block or bradycardia with atrial fibrillation, baseline LV ejection fraction (LVEF) ≥ 50%, and echocardiogram recorded at least 6 months postimplantation were included. The paced QRS recorded immediately after implantation was analyzed. A CLBBB-like paced QRS was defined as meeting the CLBBB criteria of the American Heart Association/American College of Cardiology Foundation/Heart Rhythm Society in 2009. PICM was defined as a ≥10% LVEF decrease, resulting in an LVEF of <50%. RESULTS: Among the 270 patients analyzed, PICM was observed in 38. Baseline LVEF was lower in patients with PICM, and CLBBB-like paced QRS was frequently observed in PICM. Multivariate analysis revealed that low baseline LVEF (odds ratio [OR]: 0.93 per 1% increase, 95% confidence interval [CI]: 0.89-0.98, p = 0.006) and CLBBB-like paced QRS (OR: 2.69, 95% CI: 1.25-5.76, p = 0.011) were significantly associated with PICM development. CONCLUSION: CLBBB-like paced QRS may be a novel risk factor for PICM. RV pacing, which causes CLBBB-like QRS morphology, may need to be avoided, and patients with CLBBB-like paced QRS should be followed-up carefully.


Asunto(s)
Potenciales de Acción , Bloqueo de Rama , Estimulación Cardíaca Artificial , Cardiomiopatías , Electrocardiografía , Frecuencia Cardíaca , Valor Predictivo de las Pruebas , Volumen Sistólico , Función Ventricular Izquierda , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Bloqueo Atrioventricular/fisiopatología , Bloqueo Atrioventricular/diagnóstico , Bloqueo Atrioventricular/terapia , Bloqueo Atrioventricular/etiología , Bloqueo de Rama/fisiopatología , Bloqueo de Rama/diagnóstico , Bloqueo de Rama/terapia , Bloqueo de Rama/etiología , Estimulación Cardíaca Artificial/efectos adversos , Cardiomiopatías/fisiopatología , Cardiomiopatías/etiología , Cardiomiopatías/terapia , Cardiomiopatías/diagnóstico , Diagnóstico Diferencial , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Factores de Tiempo , Resultado del Tratamiento , Función Ventricular Derecha
2.
Europace ; 24(4): 587-597, 2022 04 05.
Artículo en Inglés | MEDLINE | ID: mdl-34543395

RESUMEN

AIMS: A high-density pace-mapping can depict an abrupt transition in paced QRS morphology from a poor to excellent match, unmasking the critical component of ventricular tachycardia (VT) isthmus from the entrance to exit. We sought to assess pace-mapping at multiple sites within the endo- and epicardial scars to identify the VT isthmus in patients with ischaemic (ICM) and non-ischaemic cardiomyopathy (NICM). METHODS AND RESULTS: Colour-coded maps correlating to the percentage matches between 12-lead electrocardiograms during VT and pace-mapping [referred to as correlation score maps (CSMs)] were analysed. We studied 115 CSMs (80 endo- and 35 epicardial CSMs) in 37 patients (17 ICM, 20 NICM). The CSM with an abrupt change (AC) in pacemap score (AC-type) on the endocardium was more frequently observed in ICM than in NICM [11/39 (28%) vs. 1/41 (2%); P = 0.001]. Among 35 CSMs that were analysed by the combined endo- and epicardial mapping, 10 (29%) CSMs exhibited non-AC-type on the endocardium; however, AC-type was present on the opposite epicardium. Although 24 (69%) CSMs did not show AC-type on both the endocardium and epicardium, 16 of them had either an excellent (>90%) or poor (<0%) correlation score on either side, associated with isthmus exit or entrance, respectively. However, the remaining eight CSMs had neither excellent nor poor scores. CONCLUSION: The CSM may provide electrophysiological information to localize the endo- and epicardial VT isthmus. The absence of AC-type CSM on the endocardium, which is frequently observed in NICM, appears to indicate the sub-epicardial or intramural course of the critical isthmus.


Asunto(s)
Cardiomiopatías , Ablación por Catéter , Isquemia Miocárdica , Taquicardia Ventricular , Cardiomiopatías/complicaciones , Cardiomiopatías/diagnóstico , Endocardio , Mapeo Epicárdico , Humanos , Isquemia Miocárdica/complicaciones , Isquemia Miocárdica/diagnóstico , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiología , Taquicardia Ventricular/cirugía
3.
Eur Heart J ; 42(29): 2854-2863, 2021 07 31.
Artículo en Inglés | MEDLINE | ID: mdl-34219138

RESUMEN

AIMS: The prognostic value of genetic variants for predicting lethal arrhythmic events (LAEs) in Brugada syndrome (BrS) remains controversial. We investigated whether the functional curation of SCN5A variations improves prognostic predictability. METHODS AND RESULTS: Using a heterologous expression system and whole-cell patch clamping, we functionally characterized 22 variants of unknown significance (VUSs) among 55 SCN5A mutations previously curated using in silico prediction algorithms in the Japanese BrS registry (n = 415). According to the loss-of-function (LOF) properties, SCN5A mutation carriers (n = 60) were divided into two groups: LOF-SCN5A mutations and non-LOF SCN5A variations. Functionally proven LOF-SCN5A mutation carriers (n = 45) showed significantly severer electrocardiographic conduction abnormalities and worse prognosis associated with earlier manifestations of LAEs (7.9%/year) than in silico algorithm-predicted SCN5A carriers (5.1%/year) or all BrS probands (2.5%/year). Notably, non-LOF SCN5A variation carriers (n = 15) exhibited no LAEs during the follow-up period. Multivariate analysis demonstrated that only LOF-SCN5A mutations and a history of aborted cardiac arrest were significant predictors of LAEs. Gene-based association studies using whole-exome sequencing data on another independent SCN5A mutation-negative BrS cohort (n = 288) showed no significant enrichment of rare variants in 16 985 genes including 22 non-SCN5A BrS-associated genes as compared with controls (n = 372). Furthermore, rare variations of non-SCN5A BrS-associated genes did not affect LAE-free survival curves. CONCLUSION: In vitro functional validation is key to classifying the pathogenicity of SCN5A VUSs and for risk stratification of genetic predictors of LAEs. Functionally proven LOF-SCN5A mutations are genetic burdens of sudden death in BrS, but evidence for other BrS-associated genes is elusive.


Asunto(s)
Síndrome de Brugada , Síndrome de Brugada/genética , Humanos , Mutación/genética , Canal de Sodio Activado por Voltaje NAV1.5/genética , Fenotipo , Virulencia
4.
J Cardiovasc Electrophysiol ; 31(5): 1187-1194, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-32128918

RESUMEN

INTRODUCTION: The effectiveness of rate-modulated pacing for the suppression of atrial fibrillation (AF) is controversial. Closed-loop stimulation (CLS) is a heart rate modulation technique based on the contractility of the right ventricle estimated by sensing myocardial impedance, and CLS can still adapt to the heart rate in conditions where there are no significant changes in acceleration or ventilation, such as emotional stress. We elucidated the association between CLS and atrial tachyarrhythmia (AT) burden in patients with sinus node dysfunction and paroxysmal AF history before pacemaker implantation. METHODS AND RESULTS: We retrospectively reviewed all consecutive patients who underwent pacemaker implantation for sinus node dysfunction with an AF history before implantation. Overall, 146 patients were analyzed, with fixed-rate pacing (FP) in 82, CLS in 31, and non-CLS rate modulation in 33 patients. The AF/AT episodes were detected in 98 patients during a 12-month period. The median AF/AT burden was 1.6% (interquartile: 0.0%, 11.0%) in FP; 0% (0.0%, 2.5%) in CLS, and 1.0% (0.1%, 9.3%) in non-CLS. The AF/AT burden was significantly lower for CLS than for FP and non-CLS rate modulation (P < .01 and P = .04, respectively). CLS was associated with lower risks of AF/AT occurrence (hazard ratio [HR], 0.31; P = .02) and AF/AT burden more than 5% (HR, 0.28; P = .05), even after adjusting for potential confounders. This association was independent of the percentage of atrial pacing. CONCLUSION: CLS was associated with lower AF/AT burden after pacemaker implantation in patients with sinus node dysfunction and AF history.


Asunto(s)
Fibrilación Atrial/terapia , Estimulación Cardíaca Artificial , Marcapaso Artificial , Síndrome del Seno Enfermo/terapia , Taquicardia Supraventricular/terapia , Potenciales de Acción , Anciano , Anciano de 80 o más Años , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/fisiopatología , Femenino , Frecuencia Cardíaca , Humanos , Masculino , Contracción Miocárdica , Ventilación Pulmonar , Estudios Retrospectivos , Síndrome del Seno Enfermo/diagnóstico , Síndrome del Seno Enfermo/fisiopatología , Taquicardia Supraventricular/diagnóstico , Taquicardia Supraventricular/fisiopatología , Factores de Tiempo , Resultado del Tratamiento , Función Ventricular Derecha
5.
J Cardiovasc Electrophysiol ; 27(12): 1448-1453, 2016 12.
Artículo en Inglés | MEDLINE | ID: mdl-27593399

RESUMEN

INTRODUCTION: Recent studies showed that J waves were associated with higher incidence of ventricular tachyarrhythmia (VT/VF) in patients with idiopathic ventricular fibrillation (VF) and myocardial infarction. We sought to assess the association between J waves and VT/VF in patients with nonischemic cardiomyopathy (NICM). METHODS AND RESULTS: We retrospectively enrolled 109 patients (79 men; mean age, 60 ± 15 years) with NICM who underwent implantable cardioverter defibrillator (ICD) implantation. The primary endpoint of this study was the occurrence of appropriate device therapy due to sustained VT/VF. The J wave was electrocardiographically defined as an elevation of the terminal portion of the QRS complex of >0.1 mV in at least 2 contiguous inferior or lateral leads. Among the 109 patients, 37 (34%) experienced an episode of appropriate device therapy during a median follow-up period of 25.9 (IQR 11.5-54.3) months. Kaplan-Meier curves showed that the presence of J waves on the 12-lead ECG obtained before device implantation was associated with an increased occurrence of appropriate device therapy (P < 0.001). Multivariate Cox proportional regression analysis revealed that the presence of J waves (HR 2.95; 95% CI 1.31-6.64; P = 0.009) was an independent predictor for the occurrence of appropriate device therapy. In the subgroup analysis of the patients with dilated or hypertrophic cardiomyopathy, J wave tended to increase the occurrence of appropriate device therapy (P = 0.056 and P = 0.092, respectively). CONCLUSIONS: The presence of J waves was an independent predictor for the occurrence of appropriate device therapy in patients with NICM who underwent ICD implantation.


Asunto(s)
Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Hipertrófica/complicaciones , Ventrículos Cardíacos/fisiopatología , Taquicardia Ventricular/etiología , Fibrilación Ventricular/etiología , Potenciales de Acción , Anciano , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Hipertrófica/diagnóstico , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/terapia , Desfibriladores Implantables , Cardioversión Eléctrica/instrumentación , Electrocardiografía , Femenino , Frecuencia Cardíaca , Humanos , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Análisis Multivariante , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de Riesgo , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/terapia , Factores de Tiempo , Resultado del Tratamiento , Fibrilación Ventricular/diagnóstico , Fibrilación Ventricular/fisiopatología , Fibrilación Ventricular/terapia
6.
JACC Clin Electrophysiol ; 10(1): 1-12, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37855774

RESUMEN

BACKGROUND: There are few data on ventricular fibrillation (VF) initiation in patients with inferolateral J waves. OBJECTIVES: This multicenter study investigated the characteristics of triggers initiating spontaneous VF in inferolateral J-wave syndrome. METHODS: A total of 31 patients (age 37 ± 14 years, 24 male) with spontaneous VF episodes associated with inferolateral J waves were evaluated to determine the origin and characteristics of triggers. The J-wave pattern was recorded in inferior leads in 11 patients, lateral leads in 3, and inferolateral leads in 17. RESULTS: The VF triggers (n = 37) exhibited varying QRS durations (176 ± 21 milliseconds, range 119-219 milliseconds) and coupling intervals (339 ± 46 milliseconds, range 250-508 milliseconds) with a right (70%) or left (30%) bundle branch block (BBB) pattern. Trigger patterns were associated with J-wave location: left BBB triggers with inferior J waves and right BBB triggers with lateral J waves. Electrophysiologic study was performed for 22 VF triggers in 19 patients. They originated from the left or right Purkinje system in 6 and from the ventricular myocardium in 10 and were undetermined in 6. Purkinje vs myocardial triggers showed distinct electrocardiographic characteristics in coupling interval and QRS-complex duration and morphology. Abnormal epicardial substrate associated with fragmented electrograms was identified in 9 patients, with triggers originating from the same region in 7 patients. Catheter ablation resulted in VF suppression in 15 patients (79%). CONCLUSIONS: VF initiation in inferolateral J-wave syndrome is associated with significant individual heterogeneity in trigger characteristics. Myocardial triggers have electrocardiographic features distinct from Purkinje triggers, and their origin often colocalizes with an abnormal epicardial substrate.


Asunto(s)
Síndrome de Brugada , Fibrilación Ventricular , Humanos , Masculino , Adulto Joven , Adulto , Persona de Mediana Edad , Electrocardiografía/métodos , Trastorno del Sistema de Conducción Cardíaco , Ventrículos Cardíacos
7.
Circ J ; 77(4): 968-74, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-23238367

RESUMEN

BACKGROUND: Idiopathic ventricular fibrillation (VF) or polymorphic ventricular tachycardia (PVT) arising from the right ventricular outflow tract (RVOT) is occasionally observed. The difference in the initial ventricular premature contraction (VPC) between VF/PVT and monomorphic VT (MVT) from the RVOT, however, has not yet been fully investigated. METHODS AND RESULTS: The electrocardiogram findings and the clinical characteristics were compared between 14 patients with PVT and 77 with MVT. The episodes of syncope were more frequent in the VF and/or PVT group (57%) than in the MVT group (10%). An initial VPC with a positive QRS complex in lead I was observed in 10 (71%) of 14 patients with VF/PVT, and in 27 (35%) of 77 patients with MVT (P<0.05). Although radiofrequency (RF) catheter ablation targeting the trigger VPC often produced a morphological change, VF/PVT was eliminated in 13 (93%) of 14 patients after additional RF applications. CONCLUSIONS: Malignant arrhythmias from the RVOT, although rare, should be considered when the patient has a syncopal episode and VPC with a positive QRS complex in lead I.


Asunto(s)
Electrocardiografía , Taquicardia Ventricular/fisiopatología , Adulto , Femenino , Ventrículos Cardíacos/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Taquicardia Ventricular/patología
8.
J Cardiol Cases ; 26(4): 260-263, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-36187318

RESUMEN

The efficacy of implantable cardioverter defibrillators (ICDs) for secondary prevention in spontaneous coronary artery dissection (SCAD) with ventricular fibrillation (VF) remains unclear. Herein, we report two cases of SCAD. In both cases, VF and ST-elevation myocardial infarction (STEMI) were noted, which were previously reported to increase the risk of VF recurrence and sudden cardiac death (SCD). Hence, a subcutaneous (S)-ICD was implanted for secondary prevention in each case. Previous studies have suggested that among patients with SCAD, those with a history of VF and left ventricular ejection fraction (LVEF) of <50% are at a higher risk of ventricular tachycardia or VF recurrence, whereas those with a history of smoking, STEMI, onset during pregnancy, recurrent SCAD, LVEF <50%, and left coronary artery main trunk lesion or proximal lesion are at a higher risk of SCD. Moreover, S-ICD is associated with fewer complications than transvenous-ICD, and the rate of inappropriate shock is decreasing. Therefore, risk stratification and consideration of S-ICD implantation in high-risk patients may be an important therapeutic strategy for the secondary prevention of SCAD. Learning objective: Previous studies have not shown the efficacy of implantable cardioverter defibrillators (ICDs) for spontaneous coronary artery dissection (SCAD). Meanwhile, it was also suggested that patients with SCAD, including those with a history of ventricular fibrillation (VF) and ST-elevation myocardial infarction, are at high risk of VF recurrence or sudden cardiac death. For the secondary prevention of SCAD with VF, subcutaneous ICD implantation in high-risk patients may be an important strategy.

9.
J Cardiol Cases ; 26(3): 212-216, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-36091615

RESUMEN

Anamorelin is prescribed for cancer cachexia treatment. Anamorelin is a ghrelin receptor antagonist and exerts a sodium channel blockade effect, possibly inducing disorders of the cardiac conduction system. We herein report two cases of wide QRS complex tachycardia caused by anamorelin. In both cases, the patients had liver dysfunction. Anamorelin is mainly metabolized in the liver; hence, sodium channel blockade by anamorelin during liver dysfunction can cause serious side effects, including wide QRS complex tachycardia, similar to flecainide toxicity. The differential diagnosis of wide QRS tachycardia caused by anamorelin can be challenging because conventional electrocardiogram criteria cannot be applicable in patients with drug intoxication. It can worsen the situation for the use of antiarrhythmic drugs for wide QRS tachycardia. The appropriate treatment is supportive care until anamorelin is metabolized. To our best knowledge, this is the first study to report the life-threatening adverse effects of anamorelin. Learning objective: Anamorelin is prescribed for cancer cachexia treatment. Anamorelin can cause wide QRS complex tachycardia. Our findings in the two cases we encountered indicate that we should be aware of wide QRS complex tachycardia in patients taking anamorelin, especially if they have liver dysfunction. We should suspect the condition to be the adverse effect of anamorelin and monitor the electrocardiogram and blood test findings regularly to prevent this fatal side effect.

10.
J Arrhythm ; 38(6): 1070-1079, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36524034

RESUMEN

Background: Focal Purkinje ventricular arrhythmias (VAs) might originate from the vicinity of the proximal portion of the cardiac conducting system. This study aimed to clarify the features associated with focal Purkinje VAs originating from the proximal conduction system. Methods: A total of 18 patients with focal Purkinje VAs undergoing radiofrequency catheter ablation (RFCA) were retrospectively examined and divided into the proximal type or the non-proximal type. The proximal type was defined as having the origin at the proximal half of the interventricular septum, or the proximal half and the septal side of the anterior wall. The 12-lead electrocardiogram and electrophysiological findings were investigated. Results: Seven patients met criteria for proximal type of focal Purkinje VA. Out of the 7, 4 patients with proximal VAs had multiple QRS morphologies of VAs clinically, whereas out of 11 patients with non-proximal VAs, only 1 had multiple morphologies (p = .047). VA QRS duration was shorter in the proximal type than in the non-proximal type (111.2 ± 19.8 ms vs. 135.7 ± 17.7 ms; p = .003). The absolute axis difference between sinus rhythm and VA was smaller in the proximal type (80.4 ± 46.1°vs. 138.8 ± 59.6°; p = .014). The absolute axis difference ≤134° was useful in distinguishing the two types. Recurrence of VA was recorded in 3 proximal type patients and 3 non-proximal type patients. No procedure-related conduction block was observed. Conclusion: A VA of absolute axis difference ≤134°, and multiple QRS morphologies of clinical VAs indicate a proximal origin. Focal Purkinje VAs from proximal origins can be suppressed by RFCA without severe conduction disturbance.

11.
Clin Case Rep ; 9(4): 1968-1972, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33936624

RESUMEN

During an initial diagnosis of IVF, an arrhythmic substrate may be missed for several reasons such as lack of information; thus, a careful follow-up is important. A three-dimensional mapping may identify a possible missed arrhythmic substrate in IVF.

12.
JACC Clin Electrophysiol ; 7(10): 1297-1308, 2021 10.
Artículo en Inglés | MEDLINE | ID: mdl-34217659

RESUMEN

OBJECTIVES: This study investigates the effect of stellate ganglion (SG) phototherapy in healthy participants and assesses its efficacy in suppressing electrical storm (ES) refractory to antiarrhythmic drugs and catheter ablation. BACKGROUND: Modulation of the autonomic nervous system has been shown to be an effective adjunctive therapy for ES. METHODS: Ten-minute SG phototherapy was performed twice weekly for 4 weeks in 20 healthy volunteers. To evaluate the acute and chronic effects of SG phototherapy, heart rate variability and serum concentrations of adrenaline, noradrenaline, and dopamine were obtained before phototherapy, immediately after the first phototherapy session, after 8 sessions of phototherapy, and 3 months after the first phototherapy session. In addition, the efficacy of SG phototherapy was evaluated in 11 patients with ES refractory to medication, sedation, and catheter ablation. RESULTS: In healthy participants, serum adrenaline concentration significantly decreased after phototherapy, whereas low-frequency power/high-frequency power significantly decreased during phototherapy. Moreover, the effect on heart rate variability did not last beyond 3 months. In the clinical pilot study, 7 patients had a suppression of ES after SG phototherapy; however, without maintenance therapy, 2 patients had a recurrence of ventricular arrhythmias. Furthermore, it did not control ES in 4 patients. CONCLUSIONS: SG phototherapy reduced sympathetic activity and may be a safe and effective adjunctive therapy to control ES in some patients, but its long-term efficacy remains unknown. Chronic phototherapy might help reduce ES recurrence.


Asunto(s)
Ganglio Estrellado , Taquicardia Ventricular , Arritmias Cardíacas , Humanos , Rayos Láser , Fototerapia , Proyectos Piloto
13.
Circ Arrhythm Electrophysiol ; 13(10): e008712, 2020 10.
Artículo en Inglés | MEDLINE | ID: mdl-32755394

RESUMEN

BACKGROUND: Mutations in the nuclear envelope genes encoding LMNA and EMD are responsible for Emery-Dreifuss muscular dystrophy. However, LMNA mutations often manifest dilated cardiomyopathy with conduction disturbance without obvious skeletal myopathic complications. On the contrary, the phenotypic spectrums of EMD mutations are less clear. Our aims were to determine the prevalence of nonsyndromic forms of emerinopathy, which may underlie genetically undefined isolated cardiac conduction disturbance, and the etiology of thromboembolic complications associated with EMD mutations. METHODS: Targeted exon sequencing was performed in 87 probands with familial sick sinus syndrome (n=36) and a progressive cardiac conduction defect (n=51). RESULTS: We identified 3 X-linked recessive EMD mutations (start-loss, splicing, missense) in families with cardiac conduction disease. All 3 probands shared a common clinical phenotype of progressive atrial arrhythmias that ultimately resulted in atrial standstill associated with left ventricular noncompaction (LVNC), but they lacked early contractures and progressive muscle wasting and weakness characteristic of Emery-Dreifuss muscular dystrophy. Because the association of LVNC with EMD has never been reported, we further genetically screened 102 LVNC patients and found a frameshift EMD mutation in a boy with progressive atrial standstill and LVNC without complications of muscular dystrophy. All 6 male EMD mutation carriers of 4 families underwent pacemaker or defibrillator implantation, whereas 2 female carriers were asymptomatic. Notably, a strong family history of stroke observed in these families was probably due to the increased risk of thromboembolism attributable to both atrial standstill and LVNC. CONCLUSIONS: Cardiac emerinopathy is a novel nonsyndromic X-linked progressive atrial standstill associated with LVNC and increased risk of thromboembolism.


Asunto(s)
Cardiomiopatías/genética , Enfermedades Genéticas Congénitas/genética , Atrios Cardíacos/anomalías , Bloqueo Cardíaco/genética , No Compactación Aislada del Miocardio Ventricular/genética , Proteínas de la Membrana/genética , Mutación , Proteínas Nucleares/genética , Accidente Cerebrovascular/etiología , Tromboembolia/etiología , Distrofia Muscular de Emery-Dreifuss Ligada a X/genética , Adolescente , Adulto , Anciano , Trastorno del Sistema de Conducción Cardíaco/complicaciones , Trastorno del Sistema de Conducción Cardíaco/diagnóstico , Trastorno del Sistema de Conducción Cardíaco/genética , Cardiomiopatías/complicaciones , Cardiomiopatías/diagnóstico , Niño , Femenino , Enfermedades Genéticas Congénitas/complicaciones , Enfermedades Genéticas Congénitas/diagnóstico , Predisposición Genética a la Enfermedad , Bloqueo Cardíaco/complicaciones , Bloqueo Cardíaco/diagnóstico , Humanos , No Compactación Aislada del Miocardio Ventricular/complicaciones , No Compactación Aislada del Miocardio Ventricular/diagnóstico , Masculino , Persona de Mediana Edad , Fenotipo , Síndrome del Seno Enfermo/complicaciones , Síndrome del Seno Enfermo/genética , Accidente Cerebrovascular/diagnóstico por imagen , Tromboembolia/diagnóstico por imagen , Distrofia Muscular de Emery-Dreifuss Ligada a X/complicaciones , Distrofia Muscular de Emery-Dreifuss Ligada a X/diagnóstico , Adulto Joven
14.
JACC Clin Electrophysiol ; 6(14): 1797-1807, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-33357576

RESUMEN

OBJECTIVES: This study sought to assess the performance of current diagnostic criteria and identify additional electrophysiological features differentiating orthodromic reciprocating tachycardia (ORT) with a concealed nodoventricular/nodofascicular (NV/NF) pathway from atrioventricular nodal re-entrant tachycardia (AVNRT). BACKGROUND: Diagnosing sustained supraventricular tachycardia (SVT) despite the occurrence of ventriculoatrial block (VAB) is challenging. METHODS: We analyzed electrograms of 25 sustained SVTs (9 NV/NF-ORTs [n = 7/2] and 16 AVNRTs) with VAB and 91 AVNRTs without VAB (for reference). RESULTS: More than 1 SVT, each with a different ventriculoatrial interval, was commonly induced in AVNRT cases (75%) but not in NV/NF-ORT cases (0%; p = 0.0005). Wenckebach VAB was common in NV/NF-ORTs (78%), but VAB patterns varied in AVNRTs. The His-His interval transiently prolonged in the following beat after the VAB in most AVNRTs but rarely did in NV/NF-ORTs (79% vs. 22%; p = 0.01). NV/NF-ORT was diagnosed by His-refractory premature ventricular contractions (n = 5) and the findings during right ventricular overdrive pacing showing an uncorrected/corrected post-pacing interval (PPI)-tachycardia cycle length (TCL) ≤115/110 ms (n = 5/5), orthodromic His capture (n = 6), and V-V-A (ventricle-ventricle-atrial response) response (n = 3). A single form of induced SVT (positive predictive value [PPV]: 69%; negative predictive value [NPV]: 100%), Wenckebach VAB (PPV: 70%; NPV: 87%), stable His-His interval despite VAB (PPV: 70%; NPV: 85%), orthodromic His capture (PPV: 100%; NPV: 97%), and V-V-A response (PPV: 100%; NPV: 95%) characterized NV/NF-ORT, and a PPI-TCL of ≤125 ms (PPV: 100%; NPV: 100%) characterized NV-ORT. CONCLUSIONS: Induction of a single SVT form, Wenckebach VAB, stable His-His interval despite VAB, orthodromic His capture, and V-V-A response appeared to discriminate NV/NF-ORT from AVNRT, with a PPI-TCL of ≤125 ms discriminating NV-ORT from NF-ORT and AVNRT.


Asunto(s)
Taquicardia por Reentrada en el Nodo Atrioventricular , Taquicardia Reciprocante , Taquicardia Ventricular , Estimulación Cardíaca Artificial , Electrocardiografía , Sistema de Conducción Cardíaco , Humanos , Taquicardia por Reentrada en el Nodo Atrioventricular/diagnóstico
15.
Pacing Clin Electrophysiol ; 32 Suppl 1: S47-51, 2009 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-19250111

RESUMEN

BACKGROUND: Template matching, a technique that examines the similarity between two QRS complexes, has not been broadly applied clinically. METHODS: The 16 patients enrolled in this study underwent radiofrequency catheter ablation (RFCA) at the site of five ventricular tachycardias (VT) and of premature ventricular contractions (PVC) arising from 25 sites in the right ventricular outflow tract (RVOT), under the guidance of conventional pace and activation mapping. After RFCA, (a) a template-matching score using a correlation coefficient, and (b) a pace-map score were calculated at 30 successful and 48 unsuccessful ablation sites. RESULTS: The template-matching score at successful ablation sites (94 +/- 4%) was significantly higher than at unsuccessful (85 +/- 9%) ablation sites (P < 0.001). A > or = 90% average matching score identified successful ablation sites with a sensitivity of 90% and specificity of 69%. While there was a significant correlation between the template-matching score and visually judged pace-map score (r = 0.63, P < 0.0001), the area under the receiver operating characteristic curve of the template matching score was larger than that of the pace-map score (0.80 vs. 0.67). CONCLUSIONS: Automated template matching was useful for localizing the optimal ablation site during RFCA of RVOT-VT/PVC.


Asunto(s)
Inteligencia Artificial , Mapeo del Potencial de Superficie Corporal/métodos , Ablación por Catéter/métodos , Reconocimiento de Normas Patrones Automatizadas/métodos , Taquicardia Ventricular/cirugía , Terapia Asistida por Computador/métodos , Obstrucción del Flujo Ventricular Externo/cirugía , Adolescente , Adulto , Anciano , Algoritmos , Humanos , Persona de Mediana Edad , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Taquicardia Ventricular/diagnóstico , Obstrucción del Flujo Ventricular Externo/diagnóstico , Adulto Joven
16.
Heart Rhythm ; 16(6): 921-927, 2019 06.
Artículo en Inglés | MEDLINE | ID: mdl-30633981

RESUMEN

BACKGROUND: Although fluoroscopy-guided right ventricular (RV) lead placement in the ventricular septum is a widely performed procedure, variation in true RV lead tip position confirmed via computed tomography (CT) and its prognostic implications in patients with atrioventricular block (AVB) are not well understood. OBJECTIVE: The purpose of this study was to evaluate the prognostic impact of CT-confirmed RV lead tip position. METHODS: We retrospectively enrolled 228 consecutive patients (age 77 ± 10 years; 125 men) with AVB who underwent fluoroscopy-guided RV septal lead implantation and thoracic CT after pacemaker implantation. Patients were classified into septal and free-wall groups according to RV lead tip position. The primary endpoint was the composite outcome of cardiac death and heart failure hospitalization. RESULTS: The RV lead tip was located at the free wall in 18 patients (8%). The primary endpoint occurred in 37 patients (16%) over median follow-up of 41 months. Electrocardiographic analysis found that R amplitude >0.53 mV in lead I was significantly predictive of free-wall pacing, with sensitivity of 70% and specificity of 77%. Multivariate Cox regression analysis demonstrated that the lead tip in the free wall (hazard ratio 2.93; 95% confidence interval 1.21-7.11; P = .018) was an independent predictor of the primary endpoint. CONCLUSION: Fluoroscopy-guided RV lead placement carries potential risk of unexpected RV free-wall pacing and may increase the risk of cardiac death and heart failure-related hospitalization in patients undergoing RV septal pacing due to AVB and receiving thoracic CT for medical reasons.


Asunto(s)
Bloqueo Atrioventricular/terapia , Estimulación Cardíaca Artificial/métodos , Electrocardiografía/métodos , Cirugía Asistida por Computador/métodos , Tomografía Computarizada por Rayos X/métodos , Anciano , Bloqueo Atrioventricular/fisiopatología , Femenino , Fluoroscopía/métodos , Humanos , Masculino , Pronóstico , Estudios Retrospectivos , Tabique Interventricular
17.
J Cardiovasc Electrophysiol ; 19(7): 681-8, 2008 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-18284499

RESUMEN

INTRODUCTION: Although successful ablation of ventricular tachycardia (VT) is feasible in arrhythmogenic right ventricular cardiomyopathy (ARVC), long-term recurrence is common. The aim of this study was to assess the usefulness of a change in the isolated delayed component (IDC) as an endpoint of the catheter ablation in ARVC. METHODS AND RESULTS: Eighteen patients (48 +/- 11 years) with ARVC were studied. Detailed endocardial mapping of the right ventricle (RV) was performed during sinus rhythm. IDCs were recorded in 16 patients and the latest IDCs were related to the VT circuit. Catheter ablation was carried out in the areas with the IDCs. At the end of the session, the IDC was electrically dissociated in one, disappeared in five, exhibited second-degree block in one, was significantly delayed (>or=50 ms) in three, and remained unchanged in six. The change in the IDC was correlated with the change in the type II/III late potentials in the signal-averaged electrocardiography (ECG) and the inducibility of the clinical VT after the ablation. During a follow-up of 61 +/- 38 months, VT recurred in six. The patients with a changed IDC had a significantly lower VT recurrence than those with no IDC or an unchanged IDC (P < 0.02). CONCLUSION: In patients with ARVC, (1) the IDCs during sinus rhythm are related to the clinical VT and can be a target for the ablation, (2) a change in the IDC can be used as an endpoint, and (3) qualitative analyses of the serial signal-averaged ECGs may be useful for the long-term follow-up.


Asunto(s)
Cardiomiopatías/etiología , Cardiomiopatías/cirugía , Ablación por Catéter/métodos , Electrocardiografía/métodos , Taquicardia Ventricular/complicaciones , Taquicardia Ventricular/cirugía , Disfunción Ventricular Derecha/etiología , Disfunción Ventricular Derecha/cirugía , Adulto , Anciano , Femenino , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Pronóstico , Resultado del Tratamiento
18.
J Cardiovasc Electrophysiol ; 19(9): 935-8, 2008 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-18399967

RESUMEN

BACKGROUND: An isolated ventricular noncompaction (IVNC) is an unclassified cardiomyopathy and, despite the increasing awareness of and interest in this disorder, the role of cardiac resynchronization therapy (CRT) remains obscure. OBJECTIVE: The purpose of this study was to clarify the long-term effect of CRT on IVNC in adult patients. METHODS: Four cases of IVNC were included in this study. Before the CRT device was implanted, all four patients (54 +/- 16-year-old, 4 males) presented with symptomatic congestive heart failure. Echocardiography revealed their systolic dysfunction and their left ventricular ejection fraction (LVEF) was 21 +/- 8%. There was also mechanical dyssynchrony observed between the LV septum and free wall area. The QRS duration was "narrow" (112 and 120 ms) in two patients. One patient had been resuscitated from ventricular fibrillation (VF) and two had nonsustained ventricular tachycardia (VT). A CRT defibrillator (CRT-D) was implanted in three patients with VT/VF and a CRT pacemaker (CRT-P) in a patient without VT/VF. The LV lead was positioned in a lateral branch of the coronary sinus where a thickened noncompacted wall existed. RESULTS: During the follow-up period (28 +/- 23 months), their congestive heart failure had improved in terms of the cardiothoracic ratio on the chest X-ray, B-type natriuretic peptide level, LV systolic dimension, and LVEF. No episodes of defibrillation shocks were observed. CONCLUSION: CRT may improve the prognosis and quality-of-life in patients with an IVNC with mechanical dyssynchrony.


Asunto(s)
Estimulación Cardíaca Artificial , Insuficiencia Cardíaca/prevención & control , Disfunción Ventricular Izquierda/prevención & control , Adulto , Humanos , Masculino , Persona de Mediana Edad , Resultado del Tratamiento
19.
Intern Med ; 57(1): 59-63, 2018 Jan 01.
Artículo en Inglés | MEDLINE | ID: mdl-29033440

RESUMEN

We describe a case of amiodarone-induced thyrotoxicosis (AIT) with cardiopulmonary arrest (CPA) in a 49-year-old woman. The patient had been treated with amiodarone for non-sustained ventricular tachycardia. Two weeks prior to her admission, she developed thyrotoxicosis and prednisolone (PSL, 30 mg daily) was administered with the continuation of amiodarone. However, she was admitted to our hospital for CPA. We performed total thyroidectomy to control her thyrotoxicosis and the pathological findings were consistent with type 2 AIT. She gradually improved and was discharged on day 84. This case demonstrates the importance of considering immediate total thyroidectomy for patients with uncontrollable AIT.


Asunto(s)
Amiodarona/efectos adversos , Amiodarona/uso terapéutico , Antiarrítmicos/efectos adversos , Antiarrítmicos/uso terapéutico , Paro Cardíaco/inducido químicamente , Taquicardia Ventricular/tratamiento farmacológico , Tirotoxicosis/inducido químicamente , Tirotoxicosis/cirugía , Femenino , Humanos , Persona de Mediana Edad , Tiroidectomía , Resultado del Tratamiento
20.
Circ Arrhythm Electrophysiol ; 11(4): e005705, 2018 04.
Artículo en Inglés | MEDLINE | ID: mdl-29654128

RESUMEN

BACKGROUND: Several conducting channels of ventricular tachycardia (VT) can be identified using voltage limit adjustment (VLA) of substrate mapping. However, the sensitivity or specificity to predict a VT isthmus is not high by using VLA alone. This study aimed to evaluate the efficacy of the combined use of VLA and fast-Fourier transform analysis to predict VT isthmuses. METHODS AND RESULTS: VLA and fast-Fourier transform analyses of local ventricular bipolar electrograms during sinus rhythm were performed in 9 postinfarction patients who underwent catheter ablation for a total of 13 monomorphic VTs. Relatively higher voltage areas on an electroanatomical map were defined as high voltage channels (HVCs), and relatively higher fast-Fourier transform areas were defined as high-frequency channels (HFCs). HVCs were classified into full or partial HVCs (the entire or >30% of HVC can be detectable, respectively). Twelve full HVCs were identified in 7 of 9 patients. HFCs were located on 7 of 12 full HVCs. Five VT isthmuses (71%) were included in the 7 full HVC+/HFC+ sites, whereas no VT isthmus was found in the 5 full HVC+/HFC- sites. HFCs were identical to 9 of 16 partial HVCs. Eight VT isthmuses (89%) were included in the 9 partial HVC+/HFC+ sites, whereas no VT isthmus was found in the 7 partial HVC+/HFC- sites. All HVC+/HFC+ sites predicted VT isthmus with a sensitivity of 100% and a specificity of 80%. CONCLUSIONS: Combined use of VLA and fast-Fourier transform analysis may be a useful method to detect VT isthmuses.


Asunto(s)
Potenciales de Acción , Técnicas Electrofisiológicas Cardíacas , Análisis de Fourier , Frecuencia Cardíaca , Ventrículos Cardíacos/fisiopatología , Taquicardia Ventricular/diagnóstico , Anciano , Anciano de 80 o más Años , Ablación por Catéter , Femenino , Ventrículos Cardíacos/cirugía , Humanos , Masculino , Persona de Mediana Edad , Infarto del Miocardio/complicaciones , Valor Predictivo de las Pruebas , Reproducibilidad de los Resultados , Estudios Retrospectivos , Taquicardia Ventricular/etiología , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/cirugía
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