Beta-Blocker Therapy for Long QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia: Are All Beta-Blockers Equivalent?

September 18, 2016—Drug therapy with beta-blockers represents the therapeutic mainstay for both LQTS and CPVT. The goal of the document is to help clinicians understand the importance of beta-blocker therapy in these two genetic disorders, as well as to provide clinical guidance on the choice of agents. Nadolol, when available, has been the preferred beta-blocker utilized by the largest LQTS/CPVT specialty centers throughout the world for the past 25 years.

This therapeutic paradigm is under threat in several countries, despite being recognized by chanelopathy experts around the world, and the drug is at risk of disappearing from the market. The impact of this development could be profound. For this reason, the Heart Rhythm Society (HRS) formed a task force to investigate the clinical implications. As part of these efforts, a survey was sent to both pediatric and adult heart rhythm specialists, largely in North America and Europe. More than 70% of respondents (n=109) use nadolol in at least 75% of their patients with LQTS. Thus, the potential lack of availability of nadolol would necessitate a major change in practice patterns.