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Case Reports
. 2024 Mar 28;19(6):2457-2463.
doi: 10.1016/j.radcr.2024.03.014. eCollection 2024 Jun.

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

Santiago Luna-Alcala  1 , Mauricio Garcia-Cardenas  2 , Enrique C Guerra  1   3 , Pavel Martinez-Dominguez  1 , Aldo Cabello-Ganem  1 , Leonardo Proaño-Bernal  1 , Cristian A Chava-Ponte  4 , Arturo Hernandez-Pacherres  4 , Nilda Espinola-Zavaleta  1
Affiliations
Case Reports

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

Santiago Luna-Alcala et al. Radiol Case Rep. .

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; Fibrosis; Implantable cardioverter defibrillator; Left ventricle; Sudden cardiac death; Ventricular tachycardia.

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Figures

Fig 1
Fig. 1
Phenotypic features of arrhythmogenic left ventricular cardiomyopathy. (A) 12-lead ECG revealing a monomorphic ventricular tachycardia. (B) ECG in sinus rhythm after successful pharmacological cardioversion, showing low QRS voltages (<0.5 mV peak to peak) in limb leads (black arrows) and inverted T-waves in the left precordial leads (V4-V6, red arrows).
Fig 2
Fig. 2
Gadolinium-based magnetic resonance imaging. End-diastolic bSSFP cine (A-C) showing biventricular enlargement. (D) T2-weighted short-axis view showing a linear hypointensity signal in the basal third of the interventricular septum (white arrows) and hyperintensity (red arrows) which likely represents edema. (E, F) LGE short-axis view showing LV ring-pattern subepicardial enhancement (white arrows) and irregular morphology of the RV free wall (red arrowheads). (G) Four-chamber view demonstrating diffuse LGE involving the subepicardial layer of the LV lateral wall and the interventricular septum (white arrows) and showing irregular morphology of the RV free wall (red arrowheads). (H) Vertical long-axis view showing subepicardial LGE at anterior LV wall (white arrows). Ao, Aorta; LA, Left atrium; LV, Left ventricle; RA, Right atrium; RV, Right ventricle.
Fig 3
Fig. 3
Chest X-ray after ICD placement. Frontal view chest radiography showing a single-chamber pacemaker with its characteristic shock coils in superior vena cava (black arrow) and another that surrounds RV lead (red arrow) in a 68-year-old man with biventricular arrhythmogenic cardiomyopathy with predominant LV involvement.
See this image and copyright information in PMC

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