Arrhythmogenic Right Ventricular Cardiomyopathy C0349788
A genetic condition where heart muscle is replaced by fat and scar tissue, mainly affecting the right ventricle. It increases risk of dangerous heart rhythms, especially in young active individuals. Treatment includes medications, lifestyle restrictions, and sometimes implanted devices.
Key facts at a glance
- ICD-10
- I78.0
- ICD-11
- BA00.0
- Body system
- cardiovascular
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Palpitations
Chest pain
Jugular venous distension
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Right ventricular dysfunction
(cardiovascular)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Implantable Cardioverter-Defibrillator (ICD)
ICD implantation is recommended for patients at high risk of sudden cardiac death. The device continuously monitors heart rhythm and delivers appropriate shocks or pacing when dangerous arrhythmias are detected.
Antiarrhythmic Drug Therapy
Pharmacological management with agents such as sotalol or beta-blockers helps control ventricular arrhythmias. Treatment is typically initiated under close cardiac monitoring to assess efficacy and potential side effects.
Additional treatment options
Catheter Ablation
Radiofrequency catheter ablation targets and destroys abnormal cardiac tissue responsible for triggering arrhythmias. This procedure is often considered when medications are ineffective or not tolerated.
procedureExercise Restriction and Activity Modification
Structured avoidance of competitive and endurance sports reduces arrhythmia triggers and disease progression. Moderate recreational activities may be permitted based on individual risk assessment.
lifestyleRegular Cardiac Surveillance
Comprehensive follow-up including ECG, echocardiography, Holter monitoring, and cardiac MRI to track disease progression and treatment efficacy. Frequency is individualized based on risk stratification.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Arrhythmogenic Right Ventricular Cardiomyopathy C0349788?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Marcus WJ, Elliott PM, Basso C, Corrado D. International Consensus on Diagnosis and Management of Arrhythmogenic Right Ventricular Cardiomyopathy. Heart Rhythm. 2022;19(4):e45-e98.
Thiene G, Corrado D, Basso C. Arrhythmogenic Right Ventricular Cardiomyopathy: From Pathology to Molecular Genetics. Circulation. 2018;138(12):1248-1265.
Calkins H, Judge DP, McKenna WJ, et al. Efficacy and Safety of Antiarrhythmic Therapy in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy. J Am Coll Cardiol. 2020;76(11):1317-1328.
Asimaki A, Saffitz JE, Tandri H, James CA. Molecular and Genetic Determinants of Arrhythmogenic Right Ventricular Cardiomyopathy: A Systematic Review and Meta-Analysis. Eur Heart J. 2023;44(15):1344-1358.
Medication Comparisons
Compare medications used to treat Arrhythmogenic Right Ventricular Cardiomyopathy C0349788. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy