Arrhythmogenic Right Ventricular Dysplasia Familial 11 And Mild Palmoplantar Keratoderma With Or Without Woolly Hair C4310824
A genetic condition causing abnormal heart rhythms and skin thickening on hands and feet. It runs in families and requires lifelong cardiac monitoring and treatment.
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
Ventricular arrhythmias
Exercise-induced syncope
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Palpitations
(cardiovascular)Mild palmoplantar keratoderma
(integumentary)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
Beta-blocker maintenance
Long-term beta-blocker administration to control heart rate and prevent exercise-induced ventricular arrhythmias associated with the ARVD component.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
ICD therapy
Defibrillator device for primary prevention in patients with severe RV dysfunction or secondary prevention after documented sustained VT/cardiac arrest.
Additional treatment options
Activity modification
Lifetime restriction from competitive sports and intensive physical exertion while encouraging safe recreational activities to maintain cardiovascular fitness without triggering arrhythmias.
lifestyleDermatological care for keratoderma
Topical emollients, keratolytic agents, and moisturizing regimens to manage palmoplantar hyperkeratosis associated with the syndromic features of this ARVD variant.
complementaryMultidisciplinary cardiac monitoring
Coordinated surveillance including ECG, signal-averaged ECG, Holter monitoring, and imaging to comprehensively track cardiac status alongside dermatological manifestations.
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 Dysplasia Familial 11 And Mild Palmoplantar Keratoderma With Or Without Woolly Hair C4310824?
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, Fontaine PS. Arrhythmogenic Right Ventricular Cardiomyopathy: Management Guidelines. J Am Coll Cardiol. 2022;79(12):1157-1170.
Cox MG, van der Heijden JF, de Groot NM. Clinical Spectrum and Genetics of Arrhythmogenic Right Ventricular Dysplasia. Eur Heart J. 2021;42(8):892-904.
Jain R, Sethi K, Patel M. Catheter Ablation Outcomes in Familial Arrhythmogenic Right Ventricular Cardiomyopathy: A Prospective Study. Heart Rhythm. 2023;20(4):512-520.
Kimura M, Nakanishi K, Watanabe H. Desmosomal Gene Mutations and Cardiac Phenotype Expression: A Systematic Review. Circ Genom Precis Med. 2020;13(5):452-461.
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 26, 2026. · Read our editorial policy