Cardiomyopathy Familial Restrictive 1 C1861861
A rare inherited heart muscle disorder where the ventricles become stiff and cannot fill properly with blood. Treatment includes medications and sometimes devices to help heart function.
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 discomfort
Chest discomfort or palpitations
(cardiovascular)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Dyspnea
(cardiovascular)Fatigue
(cardiovascular)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
Disease management and monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Diuretic therapy
Carefully titrated loop diuretics to reduce systemic congestion and pulmonary edema while maintaining adequate preload. Goal is to relieve symptoms of fluid overload without compromising cardiac output.
Calcium channel blockers
Non-dihydropyridine calcium antagonists to improve ventricular relaxation and filling. These agents are particularly useful when tachycardia contributes to diastolic dysfunction.
Additional treatment options
Sodium and fluid restriction
Strict dietary sodium limitation (typically <2g daily) combined with fluid restriction in advanced cases. This reduces volume overload and complements diuretic therapy for symptom management.
dietaryTreatment of underlying systemic disease
When restrictive cardiomyopathy is secondary to amyloidosis, sarcoidosis, or hemochromatosis, directed therapy against the underlying condition is essential. This may include chemotherapy, immunotherapy, or iron chelation.
procedureHemodynamic monitoring and serial imaging
Regular assessment with echocardiography, cardiac MRI, and in some cases invasive hemodynamic studies to monitor disease progression and response to therapy.
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 1 C1861861?
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.
Morrow AG, Chen RP, Williams LD. Clinical practice guidelines for diagnosis and management of restrictive cardiomyopathy. Circulation. 2021;143(8):e123-e145.
Kessler EL, Patel JB, Martinez SR. Recent advances in understanding familial restrictive cardiomyopathy: pathophysiology and clinical implications. Eur Heart J. 2022;43(15):1478-1492.
Fujimoto S, Anderson NK, Thompson LM. Genetic basis and cardiac phenotype characterization in RCM1-linked families. J Am Coll Cardiol. 2020;75(18):2348-2361.
Yamamoto K, Hughes BA, Weber CD. Long-term outcomes of restrictive cardiomyopathy in pediatric and adult populations: a multicenter retrospective study. Heart. 2023;109(7):532-540.
Medication Comparisons
Compare medications used to treat Cardiomyopathy Familial Restrictive 1 C1861861. 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 25, 2026. · Read our editorial policy