Ventricular predominance in biventricular arrhythmogenic cardiomyopathy: Should new subtype criteria be recognized?

Abstract

Arrhythmogenic cardiomyopathy is a biventricular disease in which the effect on the left ventricle can be either equivalent to or more severe than that on the right ventricle. It is a rare disease due to its low reported prevalence and typically becomes clinically evident during the second to fourth decade of life. It represents 4% of sudden cardiac death cases referred for autopsy and 10% of cases of unexplained cardiac arrest. We present a challenging case report of a 68-year-old man who arrived at the emergency room with chest discomfort, palpitations, and light-headedness before a syncopal episode with urinary incontinence. During monitoring, ventricular tachycardia was detected and was treated with cardioversion. However, a follow-up electrocardiogram revealed low QRS voltages in limb leads and T-wave inversion in the left precordial leads. The patient underwent a transthoracic echocardiogram and a gadolinium-based magnetic resonance imaging study to evaluate the possibility of acute decompensated heart failure. Both imaging studies revealed low ejection fraction and systolic dysfunction in both right and left ventricles. Furthermore, in the late gadolinium enhancement study, extensive left ventricular subepicardial enhancement with septal predominance in a ring pattern and an irregular morphology of the right ventricular free wall were observed. A diagnosis of biventricular arrhythmogenic cardiomyopathy was established based on the 2020 Padua Criteria. Although there is not a recognized classification within these criteria to establish its subtype, in our case there was a left ventricular predominance due to the presence of additional left ventricular categories.

Keywords

• Biventricular cardiomyopathy
• Left ventricle
• Fibrosis
• Implantable cardioverter defibrillator
• Sudden cardiac death
• Ventricular tachycardia