Symptoms
Fatigue
Shortness of breath, especially with exertion or when lying flat
Swelling in the ankles, feet, legs, and abdomen (edema)
Weakness
Cough, particularly when lying down
Ascites (fluid accumulation in the abdomen)
Chest pain
Palpitations (feeling of rapid or irregular heartbeats)
Lightheadedness or dizziness
Jugular venous distention (swollen neck veins)
Causes
The causes of restrictive cardiomyopathy are varied and, in some cases, unknown (idiopathic). Known causes include:
Infiltrative Diseases:
Amyloidosis (abnormal protein deposits)
Sarcoidosis (inflammatory disease affecting multiple organs)
Hemochromatosis (iron overload)
Storage Diseases:
Fabry disease
Glycogen storage diseases
Endomyocardial Fibrosis: Scarring of the inner lining of the heart chambers, common in tropical regions.
Radiation Therapy: Exposure to radiation, especially to the chest area.
Carcinoid Syndrome: Tumors that secrete substances that damage the heart.
Scleroderma: A connective tissue disease.
Idiopathic: In many cases, the cause is unknown.
Medicine Used
The goal of medication is to manage symptoms and improve heart function. There is no cure. Typical medications include:
Diuretics: To reduce fluid overload (e.g., furosemide, torsemide).
Beta-blockers, Calcium Channel Blockers, or Digoxin: May be used cautiously to control heart rate and improve filling time. Note: These medications must be used with caution, as they can sometimes worsen the condition.
Anticoagulants: To prevent blood clots, especially if atrial fibrillation is present (e.g., warfarin, newer oral anticoagulants).
Antiarrhythmics: To control irregular heart rhythms (e.g., amiodarone).
Medications to treat underlying conditions: Aimed at the disease causing the restrictive cardiomyopathy (e.g., chemotherapy for amyloidosis).
Precautions
Follow doctor's instructions: Strictly adhere to the prescribed medication regimen and lifestyle recommendations.
Dietary modifications: Limit sodium intake to reduce fluid retention.
Fluid restriction: May be necessary in some cases to manage fluid overload.
Regular exercise: Engage in light to moderate exercise as tolerated, under the guidance of a healthcare professional. Avoid strenuous activity that can worsen symptoms.
Monitor weight: Daily weight monitoring to detect fluid retention.
Avoid alcohol and tobacco: These can worsen heart function.
Vaccinations: Get recommended vaccinations, such as influenza and pneumonia, to prevent infections that can strain the heart.
Report changes in symptoms: Immediately report any worsening symptoms, such as increased shortness of breath, swelling, or chest pain, to your healthcare provider.
How long does an outbreak last?
Restrictive cardiomyopathy is not an infectious disease, so the concept of an "outbreak" is not applicable. It is a chronic condition that progresses over time.
How is it diagnosed?
Medical history and physical examination: The doctor will ask about symptoms, medical history, and perform a physical exam to look for signs of heart failure.
Echocardiogram: An ultrasound of the heart that can show the size and shape of the heart chambers, as well as how well the heart is pumping. It can also reveal the thickened or stiffened heart muscle characteristic of restrictive cardiomyopathy.
Electrocardiogram (ECG or EKG): Records the electrical activity of the heart and can detect abnormal heart rhythms or other signs of heart damage.
Cardiac MRI: A detailed imaging technique that can assess the structure and function of the heart. It can help identify infiltrative diseases or other causes of restrictive cardiomyopathy.
Cardiac Catheterization: Involves inserting a thin tube into a blood vessel and guiding it to the heart to measure pressures within the heart chambers.
Endomyocardial Biopsy: A small sample of heart muscle is taken and examined under a microscope. This can help identify specific causes of restrictive cardiomyopathy, such as amyloidosis or sarcoidosis.
Blood Tests: To rule out other causes of heart failure and to check for signs of underlying conditions such as hemochromatosis or thyroid disorders.
Timeline of Symptoms
The timeline of symptoms varies widely depending on the underlying cause and the individual. Symptoms often develop gradually:
Early Stages: Mild fatigue, shortness of breath with exertion. These may be subtle and easily dismissed.
Progressive Stages: Symptoms become more pronounced:
Increased fatigue and shortness of breath, even with minimal activity.
Swelling in the legs, ankles, and abdomen.
Coughing, especially at night.
Palpitations or chest pain.
Late Stages: Severe heart failure symptoms:
Shortness of breath at rest.
Significant swelling throughout the body.
Severe fatigue and weakness.
Difficulty sleeping due to breathing problems.
Important Considerations
Early diagnosis is crucial: Prompt diagnosis and treatment of underlying conditions can help slow the progression of restrictive cardiomyopathy.
Treatment is focused on symptom management: Because there is often no cure, management focuses on relieving symptoms and improving quality of life.
Regular follow-up is essential: Ongoing monitoring by a cardiologist is necessary to assess heart function and adjust treatment as needed.
Heart transplant may be an option in severe cases: For some individuals with severe restrictive cardiomyopathy, a heart transplant may be considered.
Genetic counseling: If there is a family history of cardiomyopathy, genetic counseling may be recommended.
Multidisciplinary care: Management often involves a team of healthcare professionals, including cardiologists, primary care physicians, nurses, and dietitians.