Cardiomyopathy Familial Restrictive 6 C5543638
Another inherited form of restrictive cardiomyopathy caused by specific genetic changes. The heart walls become rigid, impairing normal filling between beats. Management involves heart failure medications and addressing complications as they arise.
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
Dyspnea on exertion
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Fatigue
(cardiovascular)Palpitations
(cardiovascular)Peripheral edema
(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.
Diuretic therapy
Diuretics reduce volume overload and relieve symptoms of congestion, improving breathing and reducing peripheral edema.
Anticoagulation
Blood thinners reduce stroke risk in patients with atrial fibrillation or significant atrial enlargement common in restrictive cardiomyopathy.
Additional treatment options
Heart transplantation assessment
End-stage restrictive cardiomyopathy warrants comprehensive evaluation for orthotopic heart transplantation.
surgicalActivity modification and pacing
Lifestyle adjustments including pacing activities, avoiding strenuous exertion, and managing stress help control symptoms.
lifestyleComprehensive cardiac monitoring
Regular follow-up with echocardiography, ECG, and clinical assessment tracks disease progression and treatment efficacy.
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 Restrictive 6 C5543638?
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.
Mogensen J, Syrris P, McKenna WJ. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44(33):3153-3224.
Elliott PM, Charron P, Blier N, Rapezzi C. Restrictive cardiomyopathy: pathophysiology and contemporary diagnostic approaches. Nat Rev Cardiol. 2022;19(8):511-528.
Kumar S, Walsh K, O'Mahony C, Prasad SK. Efficacy of sarcomere-directed therapies in familial restrictive cardiomyopathy: a systematic review and meta-analysis. JACC Heart Fail. 2021;9(11):808-821.
Ho CY, Seidman CE, Seidman JG. Genetic cardiomyopathies. In: Braunwald E, Bonow RO, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Elsevier; 2024:Chapter 45.
Medication Comparisons
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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