Outcomes of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Treated With ß-Blockers.

Importance: Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) may experience life-threatening arrhythmic events (LTAEs) despite ß-blocker treatment. Further complicating management, the role of implantable cardioverter defibrillator (ICD) in CPVT is debated. Objective: To investigate the long-term outcomes of patients with RYR2 CPVT treated with ß-blockers only and the cost to benefit ratio of ICD. Design, Settings, and Participants: This prospective cohort study conducted from January 1988 to October 2020 with a mean (SD) follow-up of 9.4 (7.5) years included patients who were referred to the Molecular Cardiology Clinics of ICS Maugeri Hospital, Pavia, Italy. Participants included consecutive patients with CPVT who were carriers of a pathogenic or likely pathogenic RYR2 variant with long-term clinical follow-up. Exposures: Treatment with selective and nonselective ß-blocker only and ICD implant when indicated. Main Outcome and Measures: The main outcome was the occurrence of the first LTAE while taking a ß-blocker. LTAE was defined as a composite of 3 hard end points: sudden cardiac death, aborted cardiac arrest, and hemodynamically nontolerated ventricular tachycardia. Results: The cohort included 216 patients with RYR2 CPVT (121 of 216 female [55%], median [IQR] age 14, [9-30] years). During a mean (SD) follow-up of 9.4 (7.5) years taking ß-blockers only, 28 of 216 patients (13%) experienced an LTAE (annual rate, 1.9%; 95% CI, 1.3-2.7). In multivariable analysis, experiencing either an LTAE (hazard ratio [HR], 3.3; 95% CI, 1.2-8.9; P = .02) or syncope before diagnosis (HR, 4.5; 95% CI, 1.8-11.1; P = .001) and carrying a C-terminal domain variant (HR, 18.1; 95% CI, 4.1-80.8; P < .001) were associated with an increased LTAE risk during ß-blocker therapy only. The risk of LTAE among those taking selective ß-blockers vs nadolol was increased 6-fold (HR, 5.8; 95% CI, 2.1-16.3; P = .001). Conversely, no significant difference was present between propranolol and nadolol (HR, 1.8; 95% CI, 0.4-7.3; P = .44). An ICD was implanted in 79 of 216 patients (37%) who were followed up for a mean (SD) of 8.6 (6.3) years. At the occurrence of LTAE, ICD carriers were more likely to survive (18 of 18 [100%]) than non-ICD carriers (6 of 10 [60%]; P = .01). Conclusions and Relevance: In this cohort study, selective ß-blockers were associated with a higher risk of LTAE as compared with nadolol. Independently from treatment, LTAE and syncope before diagnosis and C-terminal domain variants identified patients at higher risk of ß-blocker failure, and the ICD was associated with reduced mortality in high-risk patients with CPVT.

Phrenic nerve displacement by intrapericardial balloon inflation during epicardial ablation of ventricular tachycardia: Four case reports.

BACKGROUND: Phrenic nerve (PN) injury is one of the recognized possible complications following epicardial ablation of ventricular tachycardia (VT). High-output pacing is a widely used maneuver to establish a relationship between the PN and the ablation catheter tip. An absence of PN capture is usually considered an indication that it is safe to ablate, and that successful ablation may be performed at adjacent sites. However, PN capture may impact the procedural outcome. Only a few cases have been reported in the literature that avoid PN injury by using different techniques. CASE SUMMARY: Three patients with a previous history of myocarditis and one patient with ischemic cardiomyopathy underwent epicardial ablation for drug-refractory VT. Before the procedure, transthoracic echocardiogram, coronary angiogram, and cardiac magnetic resonance imaging were performed on all patients. Under general anesthesia, endo/epicardial three-dimensional anatomical and substrate maps of the left ventricle were accomplished. Before radiofrequency delivery, the course of the PN was identified by provoking diaphragmatic stimulation with high-output pacing from the distal electrode of the ablation catheter. In every case, a scar region with late potentials was mapped along the PN course. After obtaining another epicardial access, a second introducer sheath was placed, and a vascular balloon catheter was inserted into the epicardial space and inflated with saline solution to separate the PN from the epicardium. Once the absence of PN capture had been proven, radiofrequency was applied to aim for complete late potential elimination and avoid VT induction. CONCLUSION: PN injury can occur as one of the complications following epicardial VT ablation procedures, and may prevent successful ablation of these arrhythmias. PN displacement by using large balloon catheters into the epicardial space seems to be feasible and reproducible, avoid procedure-related morbidity, and improve ablation success when performed in selected centers and by experienced operators.

Use of wearable cardioverter-defibrillator in association with catheter ablation for atrial fibrillation-related tachycardiomyopathy.

Implantable cardioverter-defibrillator (ICD) is an effective therapy in patients known to be at high risk for sudden cardiac death (SCD). Nevertheless, ICD implantation is not indicated in transient or reversible causes of SCD. Wearable cardioverter-defibrillator is increasingly used for SCD prevention in patients with a transient risk of ventricular arrhythmia.

Anesthetic and Perioperative Management of Patients With Brugada Syndrome.

Brugada syndrome (BrS) is an arrhythmogenic disease reported to be one among the leading causes of cardiac death in subjects under the age of 40 years. In these patients, episodes of lethal arrhythmias may be induced by several factors or situations, and for this reason, management during anesthesia and surgery must provide some precautions and drugs restrictions. To date, it is difficult to formulate guidelines for anesthetic management of patients with BrS because of the absence of prospective studies, and there is not a definite recommendation for neither general nor regional anesthesia, and there are no large studies in merit. For this reason, in the anesthesia management of patients with BrS, the decision of using each drug must be made after careful consideration and always in controlled conditions, avoiding other factors that are known to have the potential to induce arrhythmias and with a close cooperation between anesthetists and cardiologists, which is essential before and after surgery. In conclusion, given the absence of large studies in literature, we want to focus on some general rules, which resulted from case series and clinical practice, to be followed during the perioperative and anesthetic management of patients with BrS.

Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry.

OBJECTIVES: The PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) prospective registry was designed to assess the predictive accuracy of sustained ventricular tachycardia/ventricular fibrillation (VTs/VF) inducibility and to identify additional predictors of arrhythmic events in Brugada syndrome patients without history of VT/VF. BACKGROUND: Brugada syndrome is a genetic disease associated with increased risk of sudden cardiac death. Even though its value has been questioned, inducibility of VTs/VF is widely used to select candidates to receive a prophylactic implantable defibrillator, and its accuracy has never been addressed in prospective studies with homogeneous enrolling criteria. METHODS: Patients with a spontaneous or drug-induced type I electrocardiogram (ECG) and without history of cardiac arrest were enrolled. The registry included 308 consecutive individuals (247 men, 80%; median age 44 years, range 18 to 72 years). Programmed electrical stimulation was performed at enrollment, and patients were followed-up every 6 months. RESULTS: During a median follow-up of 34 months, 14 arrhythmic events (4.5%) occurred (13 appropriate shocks of the implantable defibrillator, and 1 cardiac arrest). Programmed electrical stimulation performed with a uniform and pre-specified protocol induced ventricular tachyarrhythmias in 40% of patients: arrhythmia inducibility was not a predictor of events at follow-up (9 of 14 events occurred in noninducible patients). History of syncope and spontaneous type I ECG (hazard ratio [HR]: 4.20), ventricular refractory period <200 ms (HR: 3.91), and QRS fragmentation (HR: 4.94) were significant predictors of arrhythmias. CONCLUSIONS: Our data show that VT/VF inducibility is unable to identify high-risk patients, whereas the presence of a spontaneous type I ECG, history of syncope, ventricular effective refractory period <200 ms, and QRS fragmentation seem useful to identify candidates for prophylactic implantable cardioverter defibrillator.

Natural history of Brugada syndrome: insights for risk stratification and management.

BACKGROUND: Treatment of patients with Brugada syndrome is complicated by the incomplete information on the natural history of the disease related to the small number of cases reported. Furthermore, the value of programmed electrical stimulation (PES) for risk stratification is highly debated. The objective of this study was to search for novel parameters to identify patients at risk of sudden death. METHODS AND RESULTS: Clinical data were collected for 200 patients (152 men, 48 women; age, 41+/-18 years) and stored in a dedicated database. Genetic analysis was performed, and mutations on the SCN5A gene were identified in 28 of 130 probands and in 56 of 121 family members. The life-table method of Kaplan-Meier used to define the cardiac arrest-free interval in patients undergoing PES failed to demonstrate an association between PES inducibility and spontaneous occurrence of ventricular fibrillation. Multivariate Cox regression analysis showed that after adjusting for sex, family history of sudden death, and SCN5A mutations, the combined presence of a spontaneous ST-segment elevation in leads V1 through V3 and the history of syncope identifies subjects at risk of cardiac arrest (HR, 6.4; 95% CI, 1.9 to 21; P<0.002). CONCLUSIONS: The information on the natural history of patients obtained in this study allowed elaboration of a risk-stratification scheme to quantify the risk for sudden cardiac death and to target the use of the implantable cardioverter-defibrillator.