Arrhythmogenic right ventricular cardiomyopathy characterized by recurrent syncope during exercise: A case report

Abstract

BACKGROUND Arrhythmogenic right ventricular (RV) cardiomyopathy is a rare and currently underrecognized cardiomyopathy characterized by the replacement of RV myocardium by fibrofatty tissue. It may be asymptomatic or symptomatic (palpitations or syncope) and may induce sudden cardiac death, especially during exercise. To prevent adverse events such as sudden cardiac death and heart failure, early diagnosis and treatment of arrhythmogenic RV cardiomyopathy (ARVC) are crucial. We report a patient with ARVC characterized by recurrent syncope during exercise who was successfully treated with combined endocardial and epicardial catheter ablation. CASE SUMMARY A 43-year-old man was referred for an episode of syncope during exercise. Previously, the patient experienced two episodes of syncope without a firm etiological diagnosis. An electrocardiogram obtained at admission indicated ventricular tachycardia originating from the inferior wall of the right ventricle. The ventricular tachycardia was terminated with intravenous propafenone. A repeat electrocardiogram showed a regular sinus rhythm with negative T waves and a delayed S-wave upstroke from leads V1 to V4. Cardiac magnetic resonance imaging showed RV free wall thinning, regional RV akinesia, RV dilatation and fibrofatty infiltration (RV ejection fraction of 38%). An electrophysiological study showed multiple inducible ventricular tachycardia as of a focal mechanism from the right ventricle. Endocardial and epicardial voltage mapping demonstrated scar tissue in the anterior wall, free wall and posterior wall of the right ventricle. Late potentials were also recorded. The patient was diagnosed with ARVC and treated with combined endocardial and epicardial catheter ablation with a very satisfactory follow-up result. CONCLUSION Clinicians should be aware of ARVC, and further workup, including imaging with multiple modalities, should be pursued. The combination of epicardial and endocardial catheter ablation can lead to a good outcome.

Recurrent Syncope in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy

“…Intercellular junctions are unable to adapt to any mechanical stress [ 11 ]. Some patients remain asymptomatic, while those with moderate to severe disease may exhibit symptoms such as dyspnea, palpitations, syncope, angina, edema, lightheadedness, and might even experience sudden death (Figure 2 ) [ 12 ]. Clinical manifestations typically emerge between adolescence and mid-adulthood.…”

Arrhythmogenic Right Ventricular Cardiomyopathy in a Patient Experiencing Out-of-Hospital Ventricular Fibrillation Arrest Twice: Case Report and Review of the Literature

“…Wu et al ( 2021) published a case report of a 43-year-old patient with three syncopal episodes during exercise and was found to be in VT in the emergency department and was administered intravenous propafenone to terminate his VT [11]. His repeat ECG post-VT termination showed normal sinus rhythm with negative T waves and a delayed S-wave upstroke from leads V1 to V4.…”

“…He had multiple inducible ventricular tachycardias and voltage mapping demonstrated scar tissue in the right ventricular anterior and posterior walls. He was diagnosed with ARVC and received treatment with combined catheter ablation to both endocardial and epicardial walls to achieve a satisfactory outcome [11].…”

Antiarrhythmic Drug Therapy in Arrhythmogenic Right Ventricular Cardiomyopathy

“…However, the efficacy for suppression of inducible VT was poor for the class 1c antiarrhythmics, as described above. A single case report details the efficacy of propafenone in the termination of VT with intravenous propafenone [ 35 ]. Another case report documents QRS widening in a child with ARVC following the initiation of oral propafenone [ 36 ].…”