Arrhythmogenic Right Ventricular Dysplasia 1 C1862511
The first identified genetic form of arrhythmogenic right ventricular disease. Caused by a gene variation on chromosome 14, it causes the heart's right ventricle to gradually develop fatty tissue. Patients need lifelong cardiac monitoring and treatment for abnormal heart rhythms.
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
Sudden cardiac death risk
Heart failure
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Fatigue
(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.
Primary Prevention ICD
Implantable defibrillator placement for primary prevention in patients meeting high-risk criteria such as severe RV dysfunction, NSVT, or genetic status. Device provides backup pacing and shock therapy.
Sotalol Antiarrhythmic Therapy
High-dose sotalol provides both beta-blockade and Class III antiarrhythmic effects particularly effective for ARVC-related ventricular tachycardia. Requires inpatient initiation with telemetry monitoring.
Additional treatment options
Substrate-Based Catheter Ablation
Combined voltage and activation mapping identifies abnormal substrate zones for targeted ablation. Epicardial access is frequently required due to the typical distribution of fibrofatty replacement.
procedureAdvanced Cardiac Imaging Surveillance
Serial cardiac MRI combined with echocardiography and ECG monitoring tracks structural progression and arrhythmic burden. Annual comprehensive evaluation recommended for most patients.
monitoringPsychological Support Program
Structured psychological intervention addresses anxiety, depression, and adjustment difficulties common in ARVC patients. Includes stress management, ICD anxiety desensitization, and family counseling.
complementaryWhen 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 Dysplasia 1 C1862511?
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.
Corrado D, Link MS, Calkins H. 2020 International Expert Consensus on Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. Heart Rhythm. 2020.
Basso C, Tsatsopoulou A, Thiene G. Arrhythmogenic right ventricular cardiomyopathy: what's in a name? J Cardiovasc Med. 2021.
Cadrin-Tourigny J, Bosman LP, Wang W. Efficacy of beta-blocker therapy in patients with arrhythmogenic right ventricular cardiomyopathy. JAMA Cardiol. 2022.
Orgeron GM, Te Riele A, James CA. Impact of systematic family screening in arrhythmogenic right ventricular cardiomyopathy: a meta-analysis. Circ Arrhythm Electrophysiol. 2023.
Medication Comparisons
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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