An Unusual Genetic Observation in a Case of Short-Coupled PVC-Triggered Ventricular Fibrillation

Sunandan Sikdar; Debabrata Bera; Kapil Kumawat; Arshad Jahangir; Praloy Chakraborty

doi:10.1016/j.jaccas.2022.09.018

An Unusual Genetic Observation in a Case of Short-Coupled PVC-Triggered Ventricular Fibrillation

JACC Case Rep. 2022 Nov 7;4(23):101651. doi: 10.1016/j.jaccas.2022.09.018. eCollection 2022 Dec 7.

Authors

Sunandan Sikdar¹, Debabrata Bera², Kapil Kumawat³, Arshad Jahangir⁴, Praloy Chakraborty⁵

Affiliations

¹ Narayana Multispeciality Hospital, Kolkata, India.
² Rabindranath Tagore International Institute of Cardiac Sciences, Kolkata, India.
³ Narayana Institute of Cardiac Sciences, Bangalore, India.
⁴ Aurora Center for Advanced Atrial Fibrillation Therapies, Aurora Cardiovascular & Thoracic Services, Aurora St. Luke's Medical Center, Advocate Aurora Health, Milwaukee, Wisconsin, USA.
⁵ Department of Cardiology, Toronto General Hospital, Toronto, Ontario, Canada.

Abstract

Ventricular fibrillation induced by short-coupled premature ventricular complex is an uncommon cause of cardiac arrest in young individuals with no structural heart disease. The genetic substrate of this condition is heterogeneous and remains incompletely defined. We describe a case of short-coupled premature ventricular complex-triggered ventricular fibrillation with a likely pathogenic variant in the titin (TTN) gene. (Level of Difficulty: Advanced.).

Keywords: CPVT, catecholaminergic polymorphic ventricular tachycardia; ICD, implantable cardioverter-defibrillator; LQTS, long QT syndrome; TTN, titin; VF, ventricular fibrillation; genetic disorders; sc-PVC, short-coupled premature ventricular complex; secondary prevention; ventricular fibrillation.

Publication types

Case Reports