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Objectives The purpose of this study was to compare the efficacy of beta-blockers in congenital long QT syndrome (LQTS). Background Beta-blockers are the mainstay in managing LQTS. Studies comparing the efficacy of commonly used beta-blockers are lacking, and clinicians generally assume they are equally effective. Methods Electrocardiographic and clinical parameters of 382 LQT1/LQT2 patients initiated on propranolol (n = 134), metoprolol (n = 147), and nadolol (n = 101) were analyzed, excluding patients 480 ms. None of the asymptomatic patients had BCEs. Among symptomatic patients (n = 101), 15 had BCEs (all syncopes). The QTc shortening was significantly less pronounced among patients with BCEs. There was a greater risk of BCEs for symptomatic patients initiated on metoprolol compared to users of the other 2 beta-blockers combined, after adjustment for genotype (odds ratio: 3.95, 95% confidence interval: 1.2 to 13.1, p = 0.025). Kaplan-Meier analysis showed a significantly lower event-free survival for symptomatic patients receiving metoprolol compared to propranolol/nadolol. Conclusions Propranolol has a significantly better QTc shortening effect compared to metoprolol and nadolol, especially in patients with prolonged QTc. Propranolol and nadolol are equally effective, whereas symptomatic patients started on metoprolol are at a significantly higher risk for BCEs. Metoprolol should not be used for symptomatic LQT1 and LQT2 patients. (J Am Coll Cardiol 2012;60:2092-9) (C) 2012 by the American College of Cardiology Foundation

To prospectively evaluate the effect of preoperative functional magnetic resonance (MR) imaging localization of language and motor areas on therapeutic decision making in patients with potentially resectable brain tumors.The Institutional Review Board approved this HIPAA-compliant study, and each patient gave written informed consent. Thirty-nine consecutive patients (19 male, 20 female; mean age, 42.2 years) referred for functional MR imaging for possible tumor resection were prospectively evaluated. A preoperative diagnosis of brain tumor was made in all patients. Sentence completion and bilateral hand squeeze tasks were used to map language and sensory motor areas. Neurosurgeons completed questionnaires regarding the proposed treatment plan before and after functional MR imaging and after surgery. They also gave confidence ratings for functional MR imaging results and estimated the effect on surgical time, extent of resection, and surgical approach. The effect of functional MR imaging on changes in treatment plan was assessed with the Wilcoxon signed rank test. Differences in confidence ratings between altered and unaltered treatment plans were assessed with the Mann-Whitney U test. The estimated influence of functional MR imaging on surgical time, extent of resection, and surgical approach was denoted with summary statistics.Treatment plans before and after functional MR imaging differed in 19 patients (P.05), with a more aggressive approach recommended after imaging in 18 patients. There were no significant differences in confidence ratings for functional MR imaging between altered and unaltered plans. Functional MR imaging resulted in reduced surgical time (estimated reduction, 15-60 minutes) in 22 patients who underwent surgery, a more aggressive resection in six, and a smaller craniotomy in two.Functional MR imaging enables the selection of a more aggressive therapeutic approach than might otherwise be considered because of functional risk. In certain patients, surgical time may be shortened, the extent of resection increased, and craniotomy size decreased.

The purpose of this study was to compare the efficacy of beta-blockers in congenital long QT syndrome (LQTS).Beta-blockers are the mainstay in managing LQTS. Studies comparing the efficacy of commonly used beta-blockers are lacking, and clinicians generally assume they are equally effective.Electrocardiographic and clinical parameters of 382 LQT1/LQT2 patients initiated on propranolol (n = 134), metoprolol (n = 147), and nadolol (n = 101) were analyzed, excluding patients1 year of age at beta-blocker initiation. Symptoms before therapy and the first breakthrough cardiac events (BCEs) were documented.Patients (56% female, 27% symptomatic, heart rate 76 ± 16 beats/min, QTc 472 ± 46 ms) were started on beta-blocker therapy at a median age of 14 years (interquartile range: 8 to 32 years). The QTc shortening with propranolol was significantly greater than with other beta-blockers in the total cohort and in the subset with QTc480 ms. None of the asymptomatic patients had BCEs. Among symptomatic patients (n = 101), 15 had BCEs (all syncopes). The QTc shortening was significantly less pronounced among patients with BCEs. There was a greater risk of BCEs for symptomatic patients initiated on metoprolol compared to users of the other 2 beta-blockers combined, after adjustment for genotype (odds ratio: 3.95, 95% confidence interval: 1.2 to 13.1, p = 0.025). Kaplan-Meier analysis showed a significantly lower event-free survival for symptomatic patients receiving metoprolol compared to propranolol/nadolol.Propranolol has a significantly better QTc shortening effect compared to metoprolol and nadolol, especially in patients with prolonged QTc. Propranolol and nadolol are equally effective, whereas symptomatic patients started on metoprolol are at a significantly higher risk for BCEs. Metoprolol should not be used for symptomatic LQT1 and LQT2 patients.

Initially performed for angina in the early 1900’s [1], left cardiac sympathetic denervation (LCSD) has become an important therapeutic option in patients with long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) [2-5]. For these heritable channelopathies, LCSD has filled the therapeutic gap between daily medications (i.e. beta blockers) and implantable cardioverter-defibrillator (ICD) therapy. LCSD’s anti-fibrillatory properties have been demonstrated clearly for patients with LQTS where there is an estimated 90% reduction in LQT-triggered cardiac events post-denervation [2, 4]. Hypertrophic cardiomyopathy (HCM) is one of the most prevalent heritable cardiovascular disease and the most common cause of sudden cardiac death in young athletes [6]. Clinically, HCM is characterized by unexplained and unequivocal cardiac hypertrophy in the absence of hypertrophy-inducing conditions such as hypertension and aortic stenosis. Besides the underlying genotypic heterogeneity, HCM is characterized by profound clinical heterogeneity as well [6, 7]. Patients can display extremely varying degrees of hypertrophy, fibrosis, and left ventricular outflow tract obstruction, and have an increased propensity for potentially life-threatening ventricular arrhythmias [6]. A recent article reported success using neuraxial modulation, either LCSD or thoracic epidural anesthesia (TEA), in adult patients with refractory arrhythmias, including 3 patients with HCM [8]. Herein, we report a pediatric patient with HCM who underwent LCSD for his malignant, ventricular fibrillation-prone HCM substrate, in hopes of delaying cardiac transplantation.