RBM20-Associated Ventricular Arrhythmias in a Patient with Structurally Normal Heart.
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ABSTRACT:
SUBMITTER: Vakhrushev Y
PROVIDER: S-EPMC7828370 | biostudies-literature | 2021 Jan
REPOSITORIES: biostudies-literature
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Project description:Gene mutations in RBM20 have been identified in a minority of familial and sporadic dilated cardiomyopathy cases. Recent studies of carriers of RBM20 mutations not only highlight the aforementioned association with dilated cardiomyopathy but also indicate a link with increased incidence of ventricular arrhythmias. Herein we describe a case of 17-year-old female patient with dilated cardiomyopathy carrying a p.(Arg634Trp) RBM20 mutation and presenting with frequent premature ventricular contractions and episodes of non-sustained ventricular tachycardia.
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Project description:Small-conductance Ca(2+)-activated K(+) (SK) currents are important in the repolarization of normal atrial (but not ventricular) cardiomyocytes. However, recent studies showed that the SK currents are upregulated in failing ventricular cardiomyocytes, along with increased SK channel protein expression and enhanced sensitivity to intracellular Ca(2+). The SK channel activation may be either anti-arrhythmic or pro-arrhythmic, depending on the underlying clinical situations. While the SK channel is a new target of anti-arrhythmic therapy, drug safety is still one of the major concerns.
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Project description:Congestive heart failure (HF) is a progressive affliction defined as the inability of the heart to sufficiently maintain blood flow. Ventricular arrhythmias (VAs) are common in patients with HF, and conversely, advanced HF promotes the risk of VAs. Management of VA in HF requires a systematic, multimodality approach that comprises optimization of medical therapy and use of implantable cardioverter-defibrillator and/or device combined with cardiac resynchronization therapy. Catheter ablation is one of the most important strategies with the potential to abolish or decrease the number of recurrences of VA in this population. It can be a curative strategy in arrhythmia-induced cardiomyopathy and may even save lives in cases of an electrical storm. Additionally, modulation of the autonomic nervous system and stereotactic radiotherapy have been introduced as novel methods to control refractory VAs. In patients with end-stage HF and refractory VAs, an institution of the mechanical circulatory support device and cardiac transplant may be considered. This review aims to provide an overview of current evidence regarding management strategies of VAs in HF with an emphasis on interventional treatment.
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Project description:This study tested the hypothesis that concomitant sympathetic and parasympathetic stimulation ("autonomic conflict") may act as a trigger for arrhythmias in long QT syndrome (LQTS). Studies were performed in isolated innervated rabbit hearts treated with clofilium (100 nmol/L); a potassium channel blocker. The influence of vagus nerve stimulation (VNS) on spontaneous ventricular arrhythmias was assessed in the absence/presence of sustained noradrenaline perfusion (100 nmol/L) and with sudden adrenergic stress (injections of noradrenaline into the perfusion line). Hearts were instrumented for a pseudo-electrocardiogram and monophasic action potential recordings. VNS, which slows heart rate, was associated with a stimulation frequency-dependent incidence of spontaneous early after-depolarisations (EADs) and ventricular tachycardia (VT), best predicted by the duration of the electrocardiographic T-wave and by triangulation of the ventricular action potential. In the presence of sustained (steady-state) noradrenaline perfusion, the incidence of EADs and VT with VNS was decreased from 73/55% to 45/27%, respectively. However, sudden adrenergic stress, imposed during periods of sustained VNS, was associated with a transient increase in the incidence of severity of observed arrhythmias, as indicated by an increase in the average arrhythmias score (1.6 ± 0.4 vs. 2.1 ± 0.7, p = .01). Analysis of electrophysiological parameters suggests that sudden adrenergic stress is associated with a transient prolongation, and increased triangulation, of the ventricular action potential, which may predispose to triggered activity. This study demonstrates that autonomic conflict is a pro-arrhythmic stimulus in LQTS. However, combined adrenergic and parasympathetic stimulation has a complex relationship with arrhythmogenicity, with differences in the effects of steady-state adrenergic activation vs. sudden adrenergic stress.
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Project description:IntroductionMany primary prevention heart failure (HF) patients with an implantable cardiac defibrillator (ICD) rarely experience life-threatening ventricular arrhythmias (VA). New strategies are required to identify patients most at risk of VA and sudden cardiac death who would benefit from an ICD. One potential method is the detection of fragmented QRS (fQRS) on the electrocardiogram. The aim was to assess the predictive capacity of fQRS for VA and mortality in ischemic (ICM) and non-ischemic cardiomyopathy (NICM) primary prevention HF patients.Methods and resultsA systematic review and meta-analysis of studies examining fQRS in HF patients with or without an ICD who met primary prevention indications with reduced ejection fraction ≤40%. Outcome measures were VA (or appropriate ICD therapy) and all-cause mortality. Ten studies involving 3885 patients were included for analysis. Most patients were male with non-fQRS patients being significantly younger (-1.5[-2.66, -0.42], p = .03). Diabetes was more likely in fQRS patients (1.12[1.01, 1.25], p = .03) while non-fQRS patients were 28% more likely to have a history of atrial fibrillation (0.82[0.67,1.00], p = .05). Ventricular arrhythmias were significantly 1.5 times more likely in patients with fQRS (1.51[1.02, 2.25], p = .04). HF patients were 1.7 times more likely to die of any cause if fQRS was present (1.68[1.13, 2.52], p = .01). NICM patients with fQRS have a significant 2.6-fold increased incidence of death compared with ICM patients (2.55[1.63, 3.98], p < .0001).ConclusionfQRS is associated with VA and all-cause mortality and may be a novel marker in the risk stratification of primary prevention HF patients indicated for ICD implantation.
| S-EPMC8739614 | biostudies-literature
Project description:The impact of non-sustained ventricular tachycardia (NSVT) on the risk of thromboembolic event and clinical outcomes in patients without structural heart disease remains undetermined. This study aimed to evaluate the association between NSVT and clinical outcomes.The study population of 5903 patients was culled from the "Registry of 24-hour ECG monitoring at Taipei Veterans General Hospital" (REMOTE database) between January 1, 2002 and December 31, 2004. Of that total, we enrolled 3767 patients without sustained ventricular tachycardia, structural heart disease, and permanent pacemaker. For purposes of this study, NSVT was defined as 3 or more consecutive beats arising below the atrioventricular node with an RR interval of <600 ms (>100 beats/min) and lasting < 30 seconds.There were 776 deaths, 2042 hospitalizations for any reason, 638 cardiovascular (CV)-related hospitalizations, 350 ischemic strokes, 409 transient ischemic accident (TIA), 368 new-onset heart failure (HF), and 260 new-onset atrial fibrillation (AF) with a mean follow-up duration of 10 ± 1 years. In multivariate analysis, the presence of NSVT was independently associated with death (hazard ratio [HR]: 1.362, 95% confidence interval [CI]: 1.071-1.731), CV hospitalization (HR: 1.527, 95% CI: 1.171-1.992), ischemic stroke (HR: 1.436, 95% CI: 1.014-2.032), TIA (HR 1.483, 95% CI: 1.069-2.057), and new-onset HF (HR: 1.716, 95% CI: 1.243-2.368). There was no significant association between the presence of NSVT and all-cause hospitalization or new-onset AF.In patients without structural heart disease, presence of NSVT on 24-hour monitoring was independently associated with death, CV hospitalization, ischemic stroke, TIA, and new onset heart failure.
| S-EPMC4993359 | biostudies-literature
Project description:BackgroundPostoperative arrhythmias after pediatric congenital heart disease (CHD) surgery are a known cause of morbidity and are associated with mortality. A comprehensive evaluation of early postoperative ventricular arrhythmias (VAs) after CHD surgery has not been reported.ObjectivesWe sought to determine the incidence of in-hospital VAs after CHD surgery and assess the clinical relevance of this arrhythmia during the postoperative hospital course.MethodsPatients undergoing CHD surgery at our center from September 2007 through December 2016 were prospectively enrolled. Univariate and multivariate analysis was used to assess the association between postoperative VAs and in-hospital mortality, adjusting for postoperative extracorporeal membrane oxygenation and stage 1 single ventricle palliation operations.ResultsA total of 2503 postoperative courses in 1835 patients were included. In all, 464 (18.5%) had VAs, of whom 135 (29.1%) received treatment. Monomorphic ventricular tachycardia was the most frequently treated ventricular arrhythmia (TVA; n=91 [62.3%]). TVAs were associated with increased postoperative extracorporeal membrane oxygenation (13.3% vs 5.5%; P < .001) and in-hospital mortality (14.9% vs 4.0%; P < .001). In multivariate analysis, TVA was an independent risk factor for in-hospital mortality (adjusted odds ratio 2.44; 95% confidence interval 1.21-4.92).ConclusionEarly postoperative VAs after CHD surgery are more common than previously reported. Postoperative VAs are associated with increased in-hospital mortality, and the subgroup of TVAs is an independent risk factor for in-hospital mortality.
| S-EPMC6491269 | biostudies-literature
Project description:BACKGROUND:We aimed to elucidate the long-term prognosis of nonspecific intraventricular conduction delay (NIVCD) in patients with structurally normal heart. METHODS:We included 107,838 patients (age, 52.1?±?15.5 years; men, 46.8%) who underwent electrocardiography in outpatient clinics or medical checkup (unmatched cohort). NIVCD was defined as QRS duration ?110?ms without meeting the criteria for bundle branch block. Patients with structurally normal heart and sinus rhythm were assigned to the NIVCD and normal QRS groups according to propensity score with matching variables of age, sex, hypertension, and diabetes (matched cohort 1), and additional PR interval (matched cohort 2). Baseline characteristics, electrocardiographic parameters, and clinical outcomes were compared in the unmatched cohort and the matched cohort. RESULTS:In the unmatched cohort, the frequencies of male sex and preexisting atrial fibrillation were significantly higher in the NIVCD group than in the normal QRS group. In matched cohort 1 (n?=?690), the NIVCD group exhibited significant slower sinus rate and longer PR interval than the normal QRS group. In matched cohort 2 (n?=?598), the cumulative incidence of atrial fibrillation was significantly higher in the NIVCD group than in the normal QRS group during a follow-up period of 8.8?±?2.9 years. NIVCD significantly increased the risk for AF (hazard ratio, 2.571; 95% confidence interval, 1.074-6.156; p?=?0.034). CONCLUSIONS:It is suggested that NIVCD may be associated with future occurrence of atrial fibrillation in patients with structurally normal heart and sinus rhythm.
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Project description:Introduction: Cardiac repolarization abnormalities are among the major causes of ventricular arrhythmias and sudden cardiac death. In humans, cardiac repolarization duration has a 24-h rhythm. Animal studies show that this rhythm is regulated by 24-h rhythms in ion channel function and that disruption of this rhythm leads to ventricular arrhythmias. We hypothesized that 24-h rhythms in QT duration can be used as a predictor for sudden cardiac death and are associated with ventricular arrhythmias. Secondly, we assessed a possible mechanistic explanation by studying the putative role of hERG channel dysfunction. Materials and Methods: In 2 retrospective studies, measures of the 24-h variation in the QT and QTc intervals (QT and QTc diurnality, QTd and QTcd, respectively) have been derived from Holter analyses and compared between groups: 1) 39 post-infarct patients with systolic heart failure (CHF: EF < 35%), of which 14 with, and 25 without a history of ventricular arrhythmias and 2) five patients with proven (LQTS2) and 16 with potential (Sotalol-induced) hERG channel dysfunction vs. 22 controls. Results: QTd was two-fold higher in CHF patients with a history of ventricular arrhythmias (38 ± 15 ms) compared to CHF patients without VT (16 ± 9 ms, p = 0.001). QTd was significantly increased in LQT2 patients (43 ± 24 ms) or those treated with Sotalol (30 ± 10 ms) compared to controls (21 ± 8 ms, p < 0.05 for both). Discussion: QT diurnality presents a novel clinical parameter of repolarization that can be derived from Holter registrations and may be useful for identification of patients at risk for ventricular arrhythmias.
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Project description:The improvement of surgical techniques resulted in significant life prolongation of many young patients with congenital heart disease (CHD). However, as these patients reach adulthood, their risk for late complications associated with surgery is also increased. One of the most difficult challenges associated with CHD is the high incidence of cardiac arrhythmias that arise from either the myocardial substrate created by abnormal physiology (pressure/volume changes, septal patches, and suture lines) or the presence of surgical scar. Catheter ablation is proven to be effective in treating atrial and ventricular arrhythmias in structurally normal hearts, and has also been used to treat arrhythmias in adults with congenital heart disease. In this review we provide an overview about diagnostic challenges, mapping and ablation techniques and outcome of patients undergoing transcatheter ablation procedures.
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