Peripartum Sinus Pauses in a Patient with a New Diagnosis of Congenital Long QT-2 Syndrome–A Management Dilemma

Abstract

Congenital Long QT Syndrome (LQTS) conveys risk for significant cardiac complications of ventricular arrhythmia and sudden cardiac death (SCD) [1]. Long-acting beta-blockers such as nadolol are recommended in LQTS [2]. Some patient groups are deemed to be at increased risk, for example women with a KCNH2 pathogenic variant (LQTS-2) [3]. Sinus pauses are not a recognised phenotype of LQTS-2, and their presence can complicate beta-blocker therapy, creating a management dilemma.