Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes

Abstract

This document does not address the indications of genetic testing in patients affected by inherited arrhythmias and their family members. Diagnostic, prognostic, and therapeutic implications of the results of genetic testing are also not included in this document because this topic has been covered by a recent publication 1 coauthored by some of the contributors of this consensus document, and it remains the reference text on this topic. Guidance for the evaluation of patients with idiopathic ventricular fibrillation, sudden arrhythmic death syndrome, and sudden unexplained death in infancy, which includes genetic Contents lists available at ScienceDirect

Prognostic Significance of the Sodium Channel Blocker Test in Patients With Brugada Syndrome

“…We defined spontaneous type 1 ECG as the appearance of type 1 ECG without any stress such as stress from fever or exercise. Type 1 ECG was defined as coved ST‐segment elevation ≥2 mm in at least 1 right precordial lead in the second, third, or fourth intercostal space 12. All 245 patients had spontaneous (n=181) or drug‐induced type 1 ECG (n=64).…”

Even a Pooled Analysis Does Not Resolve the Debate of Electrophysiology Testing in Brugada Syndrome

“…2 This unique interaction between channelopathy and substrate may, in part, explain the inconsistent findings reported with PES. Considering conflicting literature, [3][4][5][6][7][8][9][10][11] consensus statements suggesting a lesser role, 14 and statistical analysis of the current publication, if and how PES should be incorporated into the algorithm for managing BrS remains unsettled.…”

Management of Ventricular Arrhythmias in Suspected Channelopathies

“…Most arrhythmic events in congenital LQT1 occur during physical or emotional stress, at rest or in association with sudden auditory stimulation in LQT2, and during sleep or rest in LQT3 patients. 2 Although typical presentations can assist in raising the suspicion of LQTS, the history alone remains insufficient to diagnose the genotype and guide management.…”

“…22 The implantation of an ICD is pivotal secondary prevention in LQTS and a reasonable primary prevention approach in select cases. 2 Thoughtful ICD programming to prevent inappropriate shocks is important and usually requires a VF-only zone (detect rate, >220-240 beats per minute).…”

“…2 The majority of affected individuals typically have a personal or family history of syncope or autopsy-negative SCD in a first-or second-degree young relative. 25 SQTS is relatively rare with <60 cases reported in a recent expert consensus statement.…”