Arrhythmogenic right ventricular cardiomyopathy presenting with sustained ventricular tachycardia in an adolescent male: A case report

Priyanka P1*, S. Aravindakumar2

1Physician Assistant, Kauvery Hospital, Heart city, Trichy, Tamil Nadu

2Chief Consultant Interventional Cardiologist, Kauvery Hospital, Heart city, Trichy, Tamil Nadu

*Correspondence

Abstract

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), previously known as Arrhythmogenic Right Ventricular Dysplasia (ARVD), is a rare inherited cardiomyopathy characterized by progressive fibro fatty replacement of the right ventricular myocardium. This condition predisposes affected individuals to ventricular arrhythmias, heart failure, and sudden cardiac death, particularly in young patients. We report the case of a 15-year-old male who presented with sudden-onset breathlessness and syncope and was subsequently diagnosed with ARVC following electrocardiographic, echocardiographic, and cardiac magnetic resonance imaging findings. The patient presented with sustained ventricular tachycardia requiring DC cardioversion after failure of pharmacological conversion with amiodarone. He was stabilized with antiarrhythmic therapy and discharged with appropriate medical management and lifestyle recommendations.

Key words: Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC); Arrhythmogenic Right Ventricular Dysplasia (ARVD)

Introduction

Arrhythmogenic Right Ventricular Cardiomyopathy is a genetically determined myocardial disorder characterized by progressive loss of cardiomyocytes and replacement by fibro fatty tissue, predominantly involving the right ventricle. Structural remodeling disrupts normal electrical conduction, resulting in ventricular arrhythmias and an increased risk of sudden cardiac death. Early recognition is essential for preventing life-threatening complications.

Case Presentation

A 15-year-old male student presented with sudden onset breathlessness on 28 February 2026 and a history of one episode of syncope one week earlier. There was no history of chest pain, fever, hypertension, diabetes mellitus, prior cardiac disease, or significant family history of cardiovascular disorders.

Clinical Examination

On presentation, the patient was conscious and oriented but appeared dyspneic. Vital signs revealed an irregular pulse rate of 217 beats/minute, blood pressure of 100/70 mmHg, oxygen saturation of 95% on room air, and respiratory rate of 30 breaths/minute. Cardiovascular examination revealed normal heart sounds without murmurs, with occasional irregular rhythm.

Investigations

Electrocardiography demonstrated ventricular tachycardia with left bundle branch block morphology, T-wave inversion in leads V1–V3, and characteristic epsilon waves. Echocardiography showed dilated right atrium and right ventricle with preserved left ventricular function, moderate tricuspid regurgitation, and moderate pulmonary arterial hypertension. Cardiac MRI revealed significant right ventricular dilatation with regional akinesia/dyskinesia, findings highly suggestive of ARVC.

Diagnosis

The patient was diagnosed with Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) presenting with sustained ventricular tachycardia.

Management

Initial treatment with intravenous amiodarone failed to restore sinus rhythm. Therefore, synchronized DC cardioversion was performed successfully, resulting in conversion to normal sinus rhythm. The patient was subsequently managed with antiarrhythmic therapy, beta-blockers, and diuretics. Clinical status improved, and he was discharged in a hemodynamically stable condition.

Discharge Medications and Advice

Discharge medications included amiodarone, torsemide/spironolactone combination therapy, ranolazine, and pantoprazole. The patient was advised to avoid strenuous physical activity and weightlifting. Implantable Cardioverter-Defibrillator (ICD) therapy was recommended for consideration should recurrent symptoms or malignant arrhythmia develop.

Discussion

ARVC is an important cause of ventricular arrhythmias and sudden cardiac death in adolescents and young adults. Diagnosis is based on a combination of clinical, electrocardiographic, imaging, histopathological, and genetic criteria. Epsilon waves and right ventricular structural abnormalities remain classic diagnostic features. Cardiac MRI plays a pivotal role in identifying regional wall motion abnormalities and ventricular remodeling. Early diagnosis and risk stratification are essential to reduce arrhythmic events and mortality.

Conclusion

This case highlights the importance of considering ARVC in young patients presenting with syncope, palpitations, or ventricular tachycardia. Prompt recognition, appropriate imaging, and timely intervention can be lifesaving. Long-term follow-up is essential because of the progressive nature of the disease and the ongoing risk of ventricular arrhythmias and sudden cardiac death.

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