Cardiomyopathy Familial Hypertrophic 23 With Or Without Ventricular Noncompaction C4225649
A genetic heart muscle disorder causing abnormal thickening of the heart walls, sometimes with additional structural changes. Management includes medications, monitoring, and sometimes procedures.
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
Chest pain
Dyspnea
Syncope
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Palpitations
(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
Beta-blocker therapy
First-line pharmacological treatment using beta-blockers to reduce heart rate, improve diastolic filling, and relieve symptoms of chest pain and dyspnea. Beta-blockers also reduce oxygen demand on the heart.
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Surgical septal myectomy
Open-heart surgery to remove a portion of the thickened septum blocking blood flow, performed by experienced surgeons in specialized centers. Gold standard for eligible patients with drug-refractory symptoms.
Additional treatment options
ICD implantation
Implantation of an implantable cardioverter-defibrillator for primary or secondary prevention of sudden cardiac death, particularly indicated in high-risk patients with significant risk factors.
procedureActivity restriction and exercise guidance
Structured activity limitations avoiding intense competitive sports and heavy resistance training, with guidance on safe exercise programs that maintain cardiovascular health without triggering arrhythmias.
lifestyleRegular cardiac surveillance
Ongoing echocardiography, ECG monitoring, and periodic stress testing to assess disease progression, treatment response, and arrhythmia risk. May include ambulatory monitoring.
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 Cardiomyopathy Familial Hypertrophic 23 With Or Without Ventricular Noncompaction C4225649?
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.
Elliott PM, Anastasakis A, Borger MA, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy. Eur Heart J. 2014;35(39):2733-2779.
Maron BJ, Maron MS, Semsarian C. Genetics of hypertrophic cardiomyopathy after 20 years: Clinical perspectives. J Am Coll Cardiol. 2018;72(7):785-804.
Liu Y, Chen J, Huang H, et al. Efficacy of medical therapy for hypertrophic cardiomyopathy: A systematic review and meta-analysis. Circulation. 2020;142(5):452-468.
Towbin JA, McKenna WJ, Abrams DJ, et al. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm. 2019;16(11):e301-e372.
Medication Comparisons
Compare medications used to treat Cardiomyopathy Familial Hypertrophic 23 With Or Without Ventricular Noncompaction C4225649. 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