Wearable cardioverter defibrillators for patients with long QT syndrome.

BACKGROUND: Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy, but with the appropriate treatment strategy, such as beta-blockers, left cardiac sympathetic denervation (LCSD), and/or an implantable cardioverter defibrillator (ICD), most LQTS-triggered tragedies can be avoided. Since 2001, wearable cardioverter defibrillators (WCD:LifeVest™) have been available clinically. OBJECTIVE: Herein, we evaluated the use and outcome of WCDs in patients with LQTS. METHODS: We performed a retrospective review of 1027 patients with LQTS to identify patients who received a WCD, and collected pertinent clinical information regarding their LQTS diagnosis as well as indication and experience regarding use of the WCD. RESULTS: Overall, 10 LQTS patients (1%, 8 females, age at diagnosis 29 ±â€¯18 years, mean QTc 488 ±â€¯34 ms) were prescribed a WCD. Most common indication for WCD was as bridge to treatment during (temporary) situation of assessed high risk of sudden cardiac arrest (SCA; n = 6). The mean time of WCD use was 24 days (range 0 to 114 days). One patient (female, age 42, LQT2) received an appropriate VF-terminating shock 2 days after receiving her WCD. No inappropriate treatments or adverse events from wearing the WCD have occurred. CONCLUSIONS: A WCD can be considered in patients with LQTS deemed to be at high risk for SCA while up-titrating beta blockers, considering ICD therapy, or when navigating short term periods of increased SCA-risk, like the post-partum period in LQT2 women, ICD revision or temporary inactivation, or during short term administration of known QT prolonging medications.

Contemporary Outcomes in Patients With Long QT Syndrome.

BACKGROUND: Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy with a 1% to 5% annual risk of LQTS-triggered syncope, aborted cardiac arrest, or sudden cardiac death. OBJECTIVES: This study sought to evaluate LQTS outcomes from a single center in the contemporary era. METHODS: The authors conducted a retrospective study comprising the 606 patients with LQTS (LQT1 in 47%, LQT2 in 34%, and LQT3 in 9%) who were evaluated in Mayo Clinic's Genetic Heart Rhythm Clinic from January 1999 to December 2015. Breakthrough cardiac events (BCEs) were defined as LQTS-attributable syncope or seizures, aborted cardiac arrest, appropriate ventricular fibrillation-terminating implantable cardioverter-defibrillator shocks, and sudden cardiac death. RESULTS: There were 166 (27%) patients who were symptomatic prior to their first Mayo Clinic evaluation. Median age at first symptom was 12 years. Treatment strategies included no active therapy in 47 (8%) patients, beta-blockers alone in 350 (58%) patients, implantable cardioverter-defibrillators alone in 25 (4%) patients, left cardiac sympathetic denervation alone in 18 (3%) patients, and combination therapy in 166 (27%) patients. Over a median follow-up of 6.7 (IQR: 3.9 to 9.8) years, 556 (92%) patients have not experienced an LQTS-triggered BCE. Only 8 of 440 (2%) previously asymptomatic patients have experienced a single BCE. In contrast, 42 of 166 (25%) previously symptomatic patients have experienced ≥1 BCE. Among the 30 patients with ≥2 BCEs, 2 patients have died and 3 LQT3 patients underwent cardiac transplantation. CONCLUSIONS: Although outcomes have improved markedly, further optimization of treatment strategies is still needed given that 1 in 4 previously symptomatic patients experienced at least 1 subsequent, albeit nonlethal, LQTS-triggered cardiac event.

Quality of life after videoscopic left cardiac sympathetic denervation in patients with potentially life-threatening cardiac channelopathies/cardiomyopathies.

BACKGROUND: Left cardiac sympathetic denervation (LCSD) provides an additive or potentially alternative treatment option for patients with life-threatening cardiac channelopathies/cardiomyopathies. OBJECTIVE: We sought to examine the effects of LCSD on quality of life (QOL). METHODS: From November 2005 to May 2013, 109 patients who underwent LCSD were subsequently sent postoperative QOL surveys. RESULTS: Of 109 patients, 8 (7%) could not be contacted. Of the remaining 101 patients, 62 returned surveys (response rate 61%). There were an average of 4.1 ± 1.8 self-reported side effects immediately after LCSD. The most common anticipated side effects included unilateral hand dryness, color or temperature variance between sides of the face, and abnormal sweating. Although parent-reported pediatric physical QOL scores were lower than national norms, there were no differences in psychosocial QOL or disability scores (P = .09 and .33, respectively). QOL scores for adult patients were not significantly different from a US normative sample. Adult LCSD patients reported less disability than a US normative sample (P < .01). There was no correlation between QOL scores and the presence of anticipated side effects. However, among the subset of pediatric patients who continued to receive ventricular fibrillation-terminating implantable cardioverter-defibrillator shocks after LCSD, there was a correlation between their disability scores and the number of reported shocks (Spearman correlation = 0.56). The majority of patients/parents reported that they were very or somewhat satisfied with their surgery (or their child's surgery) and would definitely or probably recommend LCSD to another patient. CONCLUSION: Despite the anticipated side effects associated with LCSD, patients are satisfied with their surgery and indicate that they would recommend the surgery to another patient.

Competitive Sports Participation in Patients With Catecholaminergic Polymorphic Ventricular Tachycardia: A Single Center's Early Experience.

OBJECTIVES: The study sought to determine the outcomes of continued sports participation in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). BACKGROUND: Patients with CPVT are at increased risk of sudden death with exertion. Currently, CPVT patients are advised disqualification from nearly all sports in accordance with expert opinion guidelines. However, we have approached this complex issue with a shared decision making model respecting a patient's and their family's right to be a competitive athlete following institution of comprehensive CPVT-directed treatment program. METHODS: A retrospective record review was performed on patients with CPVT who were >6 years of age at the time of initial evaluation to determine their athlete status and frequency/scope of subsequent CPVT-triggered events. RESULTS: Among 63 eligible patients with CPVT (34 females, mean age at diagnosis 16.6 ± 12.9 years), 31 (49%) were athletes at some point in their life. Compared to the nonathletes, these athletes were significantly younger at diagnosis (11.8 ± 6.6 years vs. 21.3 ± 15.6 years; p = 0.003) and more symptomatic (21 [68%] vs. 13 [41%]; p = 0.04). Following diagnosis, 21 of 24 athletes (88%) continued competition. For these 21 athletes, 16 (76%) had experienced 32 CPVT-triggered events prior to diagnosis versus 57 events in 18 nonathletes (43%; p = 0.02). During follow-up, 3 events occurred in 3 of 21 athletes (14%) compared to 7 events in 6 of 42 nonathletes (14%, p = 1.00). No event resulted in death, and all received an adjustment in their CPVT therapy. CONCLUSIONS: Although sports participation is a risk taking behavior in undiagnosed and untreated CPVT, the risk may be acceptable for a well-treated and well-informed athlete following the diagnosis of CPVT.

Videoscopic left cardiac sympathetic denervation for patients with recurrent ventricular fibrillation/malignant ventricular arrhythmia syndromes besides congenital long-QT syndrome.

BACKGROUND: Treatment options for patients with recurrent ventricular arrhythmias refractory to pharmacotherapy and ablation are minimal. Although left cardiac sympathetic denervation (LCSD) is well established in long-QT syndrome, its role in non-long-QT syndrome arrhythmogenic channelopathies and cardiomyopathies is less clear. Here, we report our single-center experience in performing LCSD in this setting. METHODS AND RESULTS: In this institutional review board-approved study, we retrospectively reviewed the electronic medical records of all patients (N=91) who had videoscopic LCSD at our institution from 2005 to 2011. Data were analyzed for the subset (n=27) who were denervated for an underlying diagnosis other than autosomal dominant or sporadic long-QT syndrome. The spectrum of arrhythmogenic disease included catecholaminergic polymorphic ventricular tachycardia (n=13), Jervell and Lange-Nielsen syndrome (n=5), idiopathic ventricular fibrillation (n=4), left ventricular noncompaction (n=2), hypertrophic cardiomyopathy (n=1), ischemic cardiomyopathy (n=1), and arrhythmogenic right ventricular cardiomyopathy (n=1). Five patients had LCSD because of high-risk assessment and ß-blocker intolerance, none of whom had a sentinel breakthrough cardiac event at early follow-up. Among the remaining 22 previously symptomatic patients who had LCSD as secondary prevention, all had an attenuation in cardiac events, with 18 having no breakthrough cardiac events so far and 4 having experienced ≥1 post-LCSD breakthrough cardiac event. CONCLUSIONS: LCSD may represent a substrate-independent antifibrillatory treatment option for patients with life-threatening ventricular arrhythmia syndromes other than long-QT syndrome. The early follow-up seems promising, with a marked reduction in the frequency of cardiac events postdenervation.