Twenty-five-year-old woman with palpitations and hypertrophic cardiomyopathy

Heart Asia. 2019 Feb 23;11(1):e011174. doi: 10.1136/heartasia-2018-011174. eCollection 2019.

Abstract

Clinical introduction: A 25-year-old woman with a diagnosis of hypertrophic cardiomyopathy (HCM) and pre-excitation on ECG presented with unexplained syncope and daily palpitation. Genetic testing was positive for lysosome-associated membrane protein 2 (LAMP2) mutation which confirmed the diagnosis of Danon disease. Her younger sister was diagnosed with a similar condition and received a defibrillator implantation. Her 12-lead ECG (figure 1) and a long strip tracing (figure 2) are shown below.Figure 112-lead ECG.

Question: Where is the location of the accessory pathway and what is the next appropriate management?Anteroseptal pathway and catheter ablationMid-septal pathway and pacemaker/defibrillator implantationRight lateral pathway and catheter ablationFasciculoventricular pathway and electrophysiological studyLeft lateral pathway and electrophysiological study.

Keywords: WPW syndrome; cardiomyopathy hypertrophic; sudden cardiac death.