Arrhythmogenic right ventricular dysplasia presenting as ventricular tachycardia in an adolescent: A case report

Poony1*, Sripreethi2, Jayamenon3

1Non Critical ward Staff Nurse, Kauvery Hospital, Heartcity, Trichy, Tamil Nadu

2Nurse Educator, Kauvery Hospital, Heartcity, Trichy, Tamil Nadu

3Nursing superintendent, Kauvery Hospital, Heartcity, Trichy, Tamil Nadu

*Correspondence

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is a rare cardiomyopathy characterized by fibrofatty replacement of the right ventricular myocardium, leading to ventricular arrhythmias and sudden cardiac death. We report the case of a 15-year-old male who presented with sudden onset breathlessness and was found to have ventricular tachycardia with a heart rate of 217 bpm. Electrocardiography suggested monomorphic ventricular tachycardia. Echocardiography revealed features consistent with arrhythmogenic right ventricular dysplasia with preserved left ventricular function, moderate tricuspid regurgitation, and mild pulmonary arterial hypertension. The patient required DC cardioversion after failure to revert with antiarrhythmic therapy. He was stabilized with antiarrhythmics, diuretics, and supportive therapy. The patient improved clinically and was discharged in stable condition with advice for further cardiac evaluation including cardiac MRI.

Keywords: Arrhythmogenic right ventricular dysplasia; Ventricular tachycardia; Adolescent cardiomyopathy; DC cardioversion; Arrhythmia

Introduction

Arrhythmogenic right ventricular dysplasia (ARVD), also known as arrhythmogenic right ventricular cardiomyopathy (ARVC), is a genetically determined heart muscle disorder characterized by progressive replacement of myocardial cells with fibro fatty tissue. This condition predominantly affects the right ventricle and predisposes patients to ventricular arrhythmias and sudden cardiac death, particularly in young individuals and athletes.

The prevalence of ARVD is estimated to range from 1 in 2000 to 1 in 5000 individuals. Clinical manifestations vary widely, from asymptomatic cases to life-threatening ventricular tachyarrhythmias. Early diagnosis and management are essential to prevent adverse outcomes.

This report describes a case of ARVD in a 15-year-old adolescent presenting with ventricular tachycardia requiring urgent cardioversion.

Case presentation

A 15-year-old male presented with complaints of sudden onset breathing difficulty beginning approximately at noon on 28 February 2026. The patient initially sought care at a nearby healthcare facility and was subsequently referred to a tertiary care hospital for further management.  On admission, the patient was conscious and oriented. His heart rate was recorded at 217 beats per minute. Electrocardiography (ECG) revealed monomorphic ventricular tachycardia.  Further evaluation included echocardiography, which demonstrated:

Laboratory Investigations

The following investigations were performed to confirm the diagnosis and assess the patient’s clinical condition.

Blood Investigations

  • Complete Blood Count (CBC) – within normal limits
  • Serum Electrolytes – evaluated to rule out electrolyte imbalance
  • Renal Function Test (RFT) – normal
  • Liver Function Test (LFT) – normal
  • Cardiac enzymes (Troponin, CK-MB) – performed to rule out myocardial injury

Cardiac Investigations

Electrocardiogram (ECG): Showed monomorphic ventricular tachycardia.

ECG Report

2D Echocardiography

  • Features suggestive of arrhythmogenic right ventricular dysplasia
  • Good left ventricular function
  • Moderate tricuspid regurgitation (TR)
  • Mild pulmonary arterial hypertension (PAH)
  • Based on these findings, the patient was diagnosed with arrhythmogenic right ventricular dysplasia presenting with ventricular tachycardia.
  • Initial management included intravenous Amiodarone. However, the arrhythmia did not revert to sinus rhythm. Therefore, direct current (DC) cardioversion was performed successfully, restoring normal sinus rhythm.

Following cardioversion, the patient continued receiving:

  • Antiarrhythmic therapy
  • Intravenous diuretics
  • Oxygen supplementation
  • Proton pump inhibitor therapy
  • Supportive management

The patient stabilized gradually and supportive therapies were tapered. During hospitalization, there were no further episodes of syncope or palpitations. A cardiac MRI was advised for further evaluation of ARVD, and the report was awaited at the time of discharge. After clinical improvement, the patient was discharged in stable condition with advice for follow-up and further cardiology evaluation.

Discussion

Arrhythmogenic right ventricular dysplasia is an inherited cardiomyopathy primarily involving the right ventricle. It is characterized by progressive myocardial degeneration with fibro fatty replacement, leading to electrical instability.

Patients commonly present with:

  • Palpitations
  • Syncope
  • Ventricular tachycardia
  • Sudden cardiac arrest

In adolescents and young adults, ventricular tachycardia may be the first manifestation of the disease.

Diagnosis of ARVD is based on a combination of:

  • Electrocardiographic findings
  • Echocardiographic abnormalities
  • Cardiac MRI
  • Genetic testing
  • Family history
  • Management strategies depend on disease severity and arrhythmia burden.

Treatment options include:

  • Antiarrhythmic medications (e.g., amiodarone)
  • Electrical cardioversion for unstable arrhythmias
  • Implantable cardioverter-defibrillator (ICD) in high-risk patients
  • Lifestyle modifications including restriction of intense physical activity

In this case, the patient presented with hemodynamically significant ventricular tachycardia that required DC cardioversion after failure of pharmacological therapy. Early recognition and management were crucial in stabilizing the patient and preventing potential complications such as sudden cardiac death.

Nursing Management

  • Nursing care plays a crucial role in stabilizing the patient and preventing complications.
  • Continuous Monitoring
  • Continuous ECG monitoring to detect arrhythmia
  • Monitoring of vital signs (heart rate, blood pressure, oxygen saturation)
  • Assessment of level of consciousness
  • Medication Administration
  • Administration of prescribed antiarrhythmic drugs such as amiodarone
  • Monitoring for drug side effects and therapeutic response
  • Oxygen Therapy
  • Administer oxygen as prescribed
  • Monitor respiratory status and oxygen saturation
  • Pre- and Post-Cardioversion Care
  • Prepare the patient for DC cardioversion
  • Ensure availability of emergency equipment
  • Monitor cardiac rhythm after the procedure
  • Assess for complications such as hypotension or recurrent arrhythmia
  • Patient and Family Education
  • Educate about disease condition and treatment
  • Advise avoiding strenuous physical activity
  • Importance of regular follow-up with cardiologist
  • Inform about warning signs such as palpitations, dizziness, or syncope

Conclusion

This case highlights the importance of considering arrhythmogenic right ventricular dysplasia as a differential diagnosis in adolescents presenting with ventricular tachycardia. Prompt diagnosis and timely intervention, including electrical cardioversion and antiarrhythmic therapy, can significantly improve patient outcomes. Further evaluation with cardiac MRI and long-term follow-up is essential in managing such patients.

References

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