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Saul J.P.,Ohio State University | Schwartz P.J.,Center for Cardiac Arrhythmias of Genetic Origin | Ackerman M.J.,Molecular Therapeutics | Triedman J.K.,Harvard University
Heart Rhythm | Year: 2014

Electrocardiographic (ECG) screening in infants and children who may be at risk of sudden cardiac death (SCD) is controversial, and both rational and emotional arguments have often been given equal weight. We all have direct experience in this field, but have different backgrounds and have expressed divergent views on this topic. We attempted to build consensus among ourselves on the basis of the available facts, in the hope of providing an unbiased review of the relevant science and policy issues in favor of or against ECG screening in infants and children. This report presents our shared view on this medically and societally important topic. Long QT syndrome (LQTS) satisfies several criteria that may make ECG screening worthwhile: it is not rare (~1 in 2000 births); ECG diagnosis is feasible and can be used to trigger appropriate genetic testing; it causes approximately 10% of cases of sudden infant death syndrome (SIDS) as well as deaths in childhood and later in life, and effective treatments are available. By stimulating cascade screening in family members, diagnosis of affected infants may also prompt identification of asymptomatic but affected individuals. Neonatal screening is cost-effective by conventional criteria, and with a corrected QT (QTc) cutoff of 460 ms in 2 different ECGs, the number of false positives is estimated to be low (~1 in 1000 births). It is our conclusion that parents of newborn children should be informed about LQTS, a life-threatening but treatable disease of significant prevalence that may be diagnosed by a simple ECG. © 2014 Heart Rhythm Society. All rights reserved. Source

Schwartz P.J.,Center for Cardiac Arrhythmias of Genetic Origin
Nature Reviews Cardiology | Year: 2014

Experimental and clinical evidence indicating an antiarrhythmic effect of cardiac sympathetic denervation has been available for 100 years. Experimental data show that left cardiac sympathetic denervation (LCSD), in particular, is not only antiarrhythmic, but also antifibrillatory - an effect exquisitely important for any clinical condition associated with a high risk of ventricular fibrillation and sudden cardiac death. LCSD has additional effects on both the coronary circulation and the mechanical performance of the left ventricle, with important implications for patients with ischaemic cardiomyopathy. Evidence also shows that LCSD increases the vagal activity directed to the heart, which has potential implications for the management of heart failure. In this Review, the current and novel clinical indications for LCSD are discussed, particularly in the context of results obtained in patients with channelopathies, such as long QT syndrome and catecholaminergic polymorphic ventricular tachycardia. © 2014 Macmillan Publishers Limited. All rights reserved. Source

Schwartz P.J.,University of Pavia | Schwartz P.J.,Center for Cardiac Arrhythmias of Genetic Origin | Schwartz P.J.,University of Cape Town | Schwartz P.J.,Stellenbosch University | Schwartz P.J.,King Saud University
Swiss Medical Weekly | Year: 2013

The long QT syndrome (LQTS) is a leading cause of sudden death in the young. It is not as rare as previously assumed, given its established prevalence of 1:2,000 live births. It is characterised by prolongation of the QT interval and by the occurrence of syncope, due to torsades-despointes ventricular tachycardia, cardiac arrest and sudden death; these life-threatening cardiac events are usually, but not always, associated with physical or emotional stress. It is a genetic disorder, and knowledge of the genotype impacts significantly on management. Extremely effective therapies are available, which makes the existence of un-diagnosed affected and symptomatic patients inexcusable. Indeed, mortality for properly treated patients has now declined to around 1% over a 10-year period. This review, aimed at the clinical cardiologist, discusses briefly the essential genetic information and focuses primarily on the main issues of diagnosis and therapy. One special point of interest is in the impact of genetics on clinical management and the potential medicolegal consequences of not pursuing genetic screening in the proband and hence in the family members. Source

De Ferrari G.M.,Cardiovascular Clinical Research Center | De Ferrari G.M.,University of Pavia | Maier L.S.,University of Regensburg | Mont L.,University of Barcelona | And 17 more authors.
Heart Rhythm | Year: 2015

Background Currently available antiarrhythmic agents for the treatment of atrial fibrillation (AF) have important limitations, leaving an unmet need for safe and effective therapy. Ranolazine is an approved antianginal agent with a favorable safety profile and electrophysiologic properties suggesting a potential role in the treatment of AF. Objective The purpose of this study was to assess the safety and efficacy of ranolazine in the prevention of AF recurrence after successful electrical cardioversion and to ascertain the most appropriate dose of this agent. Methods This prospective, multicenter, randomized, double-blind, placebo-control parallel group phase II dose-ranging trial randomized patients with persistent AF (7 days to 6 months) 2 hours after successful electrical cardioversion to placebo, or ranolazine 375 mg, 500 mg, or 750 mg bid. Patients were monitored daily by transtelephonic ECG. The primary end-point was the time to first AF recurrence. Results Of 241 patients randomized, 238 took at least 1 drug dose. Ranolazine proved to be safe and tolerable. No dose of the drug significantly prolonged time to AF recurrence. AF recurred in 56.4%, 56.9%, 41.7%, and 39.7% of patients in the placebo, ranolazine 375 mg, ranolazine 500 mg, and ranolazine 750 mg groups, respectively. The reduction in overall AF recurrence in the combined 500-mg and 750-mg groups was of borderline significance compared to the placebo group (P =.053) and significant compared to 375-mg group (P =.035). Conclusion No dose of ranolazine significantly prolonged time to AF recurrence. However, the 500-mg and 750 mg-groups combined reduced AF recurrences, suggesting a possible role for this agent in the treatment of AF. © 2015 Published by Elsevier Inc. Source

De Ferrari G.M.,Cardiovascular Clinical Research Center | Schwartz P.J.,Center for Cardiac Arrhythmias of Genetic Origin | Schwartz P.J.,University of Pavia | Schwartz P.J.,University of Cape Town | And 2 more authors.
Journal of Cardiovascular Translational Research | Year: 2014

Heart failure (HF) is characterized by an autonomic imbalance with withdrawal of vagal activity and increased sympathetic activity. Novel non-pharmacological approaches to HF aimed at increasing vagal activity are being proposed. Left cardiac sympathetic denervation (LCSD) has been shown to modify favorably the outcome of several disorders characterized by life-threatening arrhythmias triggered by increased sympathetic activity. The present manuscript discusses the rationale and the limited experimental and clinical experience suggesting a potential role for LCSD in the treatment of patients with advanced heart failure. Possible future clinical applications of LCSD may include HF patients who are intolerant to β-adrenergic blockade, HF patients who have frequent implantable cardioverter-defibrillator shocks, and HF patients in countries where the likelihood of receiving a device is limited, but the capability to perform a one in a lifetime procedure is present. © 2014 Springer Science+Business Media. Source

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