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  • More recently Brugada et al used flecainide to identify the

    2019-05-17

    More recently, Brugada et al. [131]. used flecainide to identify the full extent of low-voltage electrogram activity in the anterior RV and RVOT and targeted this region for RFA. In all 14 BrS patients, RFA eliminated abnormal bipolar electrograms, normalized ST-segment elevation on right precordial leads of ECG, and VT/VF was no longer inducible. Ablation therapy can be lifesaving in otherwise uncontrollable cases. RF ablation may be considered (Class IIb recommendation) in BrS patients with frequent appropriate ICD shocks due to recurrent electrical storms [7]. There are no clinical reports of ablation of the LV substrate in patients with ERS. In patients in whom BrS combines with ERS, ablation of the anterior RV epicardium (including the RVOT) is not ameliorative.
    Pharmacologic approach to therapy
    Acknowledgments We are grateful to Buchang Pharmaceutical for providing the funds to host the J-Wave Consensus Conference. We are also grateful to Dr. Mina Chung for helpful discussions and advice and to Ms. Donna Loyle for proofing the manuscript.
    Introduction There are several options for the treatment of hereditary ventricular arrhythmias such as long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT). Administration of ß-blocker medication remains a first-line treatment; nevertheless, certain patients may be refractory or intolerant to these drugs [1–4]. Implantable cardioverter defibrillators (ICD) are often implanted in high-risk patients to prevent sudden death [1–4]. However, ICD meclofenamate in young and active patients necessitates life-long and routine device replacement and is furthermore associated with device malfunction (including inappropriate shocks), infection, and psychological problems [2,4–6]. Although ICD shocks are generally effective for ventricular fibrillation; shocks for polymorphic ventricular tachycardia (VT), bidirectional VT, and electrical storm may not be effective in terminating these tachyarrhythmias; especially in CPVT patients [2,4–8]. Therefore, the clinical gap between ß-blocker medication and ICD implantation is wide and decisions regarding the correct course of treatment are consequently challenging. Traditionally, the major clinical indications for left cardiac sympathetic denervation (LCSD) are ß-blocker intolerance or refractoriness, high risk of sudden death with ß-blocker treatment (despite the patient being asymptomatic), frequent ICD shocks, or bridging to an ICD implantation in infants and small children [2,4,6,8–14]. LCSD is especially effective in patients with poor ß-blocker compliance as it has long-lasting effects [10,11]. LCSD in patients with frequent ICD shocks significantly reduces the number of shocks, thus improving quality of life [6,10,11]. Recently, LCSD via a video-assisted thoracoscopic surgery (VATS) approach has been used in patients with LQTS and CPVT, allowing for early ambulation and short hospital stays with minimal perioperative complications [6,13–17]. Despite these advantages, LCSD is rarely used as a supplementary therapy to ß-blocker administration and ICD implantation [1–4]. LCSD prevents norepinephrine release in the heart and raises the threshold for ventricular fibrillation without impairing myocardial contractility or reducing heart rate [18–20]. Large, multicenter studies have reported consistent efficacy for LCSD with regards to decreasing cardiac events in patients with LQTS and CPVT [6,9,10]. As LCSD is not a curative treatment and does not necessarily prevent sudden death, ICD implantation is recommended for at risk patients and LCSD remains underutilized, despite the proven benefits of this procedure [4–6,9,10]. Indeed, the most recently published guidelines for treating patients with primary arrhythmia syndromes recommend LCSD as a Class IIb treatment for patients with CPVT and as Classes I and IIa for patients with LQTS [21]. Indeed; lifestyle modifications, ß-blocker medication, LCSD, and ICD implantation can all be compared to a cautious driver, anti-lock braking systems, seat belts, and air bags in modern cars, where all of these options are complementary in the prevention of traffic accident-related injuries.