From Palpitations to Prevention: Timely Recognition of Biventricular ACM Preventing Sudden Cardiac Death

TL;DR

This paper presents a case of biventricular arrhythmogenic cardiomyopathy in a young man, highlighting how early recognition prevented sudden cardiac death.

Contribution

The paper emphasizes the importance of timely diagnosis of biventricular ACM using updated criteria and appropriate interventions.

Findings

The patient exhibited nonsustained ventricular tachycardia and suspicious cardiac imaging findings consistent with biventricular ACM.

Timely intervention with ICD placement and sotalol led to a successful recovery.

Key diagnostic features included T wave inversions and reduced right ventricular systolic function.

Abstract

Sudden cardiac death (SCD) is a prevalent and significant health concern which may be preceded by palpitations and presyncope in a young patient. Of the arrhythmogenic causes of SCD, arrhythmogenic cardiomyopathy (ACM) is rare but important with a high morbidity and mortality. Here, we present a classic case of biventricular (BiV) ACM in a man in his 30s who presented with progressive palpitations and syncope found to have nonsustained ventricular tachycardia and suspicious cardiac imaging findings. Diagnosis was confirmed according to the 2024 European Task Force Criteria: T wave inversions in V1–V3 without a right bundle branch block (major criterion); regional right ventricular (RV) systolic dyskinesis with both reduced RV systolic function by CMR (RVEF 34% and normal 42%–66%); and enlarged RV by indexed RV EDV by CMR (131 mL/m2) (major criterion). LV global longitudinal strain was reduced on transthoracic echocardiography at −17% (normal more negative than −18%) (minor criterion) and T wave inversions in left precordial leads (V4–V6) (in the absence of complete LBBB) (minor criterion). With timely intervention, including ICD placement and sotalol initiation, the patient made a good recovery. This case serves as a critical reminder that recognizing the subtle yet telling signs of BiV‐ACM can mean the difference between life and sudden cardiac death.

Figures