Arrhythmogenic Cardiomyopathy With Variable Ectodermal Abnormalities C5882696
A genetic heart muscle disease causing abnormal heart rhythms along with skin and tooth abnormalities. It requires cardiac monitoring, medication, and sometimes device implantation for rhythm control.
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
Syncope
Ventricular arrhythmias
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Ectodermal abnormalities
(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
Antiarrhythmic pharmacotherapy
Standard antiarrhythmic medications such as beta-blockers or class III antiarrhythmics help control heart rhythm disturbances associated with arrhythmogenic cardiomyopathy. Treatment selection depends on specific arrhythmia patterns identified through cardiac monitoring.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Implantable cardioverter-defibrillator (ICD) placement
Device implantation for patients at high risk of sudden cardiac death due to ventricular arrhythmias. The ICD continuously monitors heart rhythm and delivers appropriate shocks or pacing when dangerous arrhythmias are detected.
Additional treatment options
Regular cardiac imaging and arrhythmia surveillance
Scheduled echocardiograms, cardiac MRI, and Holter or event monitoring to track disease progression and detect new arrhythmia formation. Allows for timely treatment adjustments based on structural and electrical changes.
monitoringSkin and ectodermal abnormality management
Dermatological treatments addressing hair, skin, and nail manifestations including specialized shampoos, topical emollients, and keratolytic agents for hyperkeratotic lesions. Coordinate with cardiology team regarding systemic retinoids which may affect cardiac function.
medicationGenetic counseling and psychosocial support
Comprehensive genetic counseling to discuss inheritance patterns and family planning implications. Psychological support addresses the emotional burden of managing a severe genetic condition affecting multiple body systems.
psychologicalWhen 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 Cardiomyopathy With Variable Ectodermal Abnormalities C5882696?
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 FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Eur Heart J. 2010.
Basso C, Corrado D, Marcus FI, et al. Arrhythmogenic right ventricular cardiomyopathy. Lancet. 2009.
Rampazzo A, Nava A, Malacrida S, et al. Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. Am J Hum Genet. 2002.
Hoffmann BA, Syed FL, Cohen IS, et al. Arrhythmogenic cardiomyopathy: clinical presentation and diagnostic approach. Curr Cardiol Rep. 2022.
Medication Comparisons
Compare medications used to treat Arrhythmogenic Cardiomyopathy With Variable Ectodermal Abnormalities C5882696. 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 20, 2026. · Read our editorial policy