Mulmodality imaging in arrhythmogenic right ventricular dysplasia: From real life to guidelines: About 18 years observation

Abstract

Introduction

ARVD has been recognized as an important cause of sudden death in association with exercise.

Objective

To determine the clinical, electrocardiographic, echocardiographic, CMR profile of ARVD in our echocardiographic laboratory.

Method

We enrolled in a prospective study from January 2005 to January 2023, 50 patients (pts) with ARVD, predominantly males (84%) mean age 32 ± 17 years old. The clinical electrocardiographic, echocardiography,cardiac magnetic resonance (CMR) if done focused on RV inflow area, the apex,the infundibulum and the left ventricule data were collected.

Results

Palpitations were reported in 30 pts (60%), syncope in 12 pts (24%). AF occurs in 4 (8%), atrial flutter in 3 pts (8.1%) with transesophageal echocardiography (TEE) in order to atrial flutter ablation. Spontaneous echo contrast in all cases and in one, left atrial appendage (LAA) thrombus. The epsilon wave was identified in 12pts (24%) and negatives T waves from V1to V3 in the absence of a complete right bundle branch block (RBBB) in 93.7% of them (Fig. 1). VT sustained and non-sustained occur in 30% and all of the pts present ventricular arrhythmia on 24-hour Holter monitoring. Echo has identify right ventricular severe dilatation and apex aneurysm with trabeculations and marked global hypokinesia in 93.7%, spontaneous echo contrast on RV in 20%, global RV dysfunction in 20 pts (54%), severe tricuspid regurgitation with marked dilatation of the tricuspid annulus in 20pts. CMR done in 21 pts shows severe RV dilatation in 20pts, RV fat infiltration in 14 pts, localized RV aneurysm in 13 pts. ICD was implanted in 10 pts and the tachycardia ventricle (VT) ablation (RFA) in 18%. CMR has confirmed 16% of biventricular ARVD. 4 patients died from respectively sudden cardiac death, after ischemic stroke and right heart failure, the third one 17 years with dilatation of the RV and thrombus died after acute occlusion of the lower extremities, the last one in the setting of digestive surgery.

Conclusion

Multimodality imaging to characterize the cardiac phenotype (morphology and function) including tissue characterization is necessary in combination with a detailed personal and family history, clinical examination, electrocardiography, echocardiography, cardiac magnetic resonance and laboratory investigations. CMR must not oppose to echocardiography but must be complementary.