Familial Hypertrophic Cardiomyopathy With Fasciculoventricular Accessory Pathway

Vikas Kalra; Krittapoom Akrawinthawong; Maitri Kalra; Rahul Jain

doi:10.1016/j.jaccas.2021.12.011

Familial Hypertrophic Cardiomyopathy With Fasciculoventricular Accessory Pathway

JACC Case Rep. 2022 Feb 16;4(4):198-204. doi: 10.1016/j.jaccas.2021.12.011.

Authors

Vikas Kalra¹, Krittapoom Akrawinthawong², Maitri Kalra¹, Rahul Jain³

Affiliations

¹ Department of Cardiology, Indiana University School of Medicine, Muncie, Indiana, USA.
² Prairie Cardiovascular, Carbondale, Illinois, USA.
³ Department of Cardiology, Indiana University School of Medicine, Indianapolis, Indiana, USA.

Abstract

Hypertrophic cardiomyopathy (HCM) is a common but an underdiagnosed condition. Fasciculoventricular bypass tract (FVBT) is rare. Concomitant presence of both conditions is well described in Danon disease. We report a case of familial HCM with FVBT linked to a heterozygous pathogenic variant, c.655G>C (p.Val219Leu), in the cardiac myosin binding protein C3 (MYBPC3) gene. (Level of Difficulty: Advanced.).

Keywords: CMR, cardiac magnetic resonance; Danon disease; ECG, electrocardiogram; FVBT, fasciculoventricular bypass tract; HCM, hypertrophic cardiomyopathy; ICD, implantable cardioverter-defibrillator; LAMP-2, lysosome-associated membrane protein 2; MYBPC3, cardiac myosin binding protein C3; NSVT, nonsustained ventricular tachycardia; WPW, Wolff-Parkinson-White syndrome; accessory pathway; familial; hypertrophic cardiomyopathy.

Publication types

Case Reports