Endomyocardial Fibrosis: Symptoms, Causes, Treatment

What are the symptoms of endomyocardial fibrosis?

Endomyocardial fibrosis (EMF) is a rare and chronic heart condition that affects the inner lining of the heart (endocardium), leading to fibrosis and thickening of the heart tissue. Symptoms often develop gradually and are related to heart failure. Common symptoms include:

Fatigue: Persistent tiredness due to reduced heart function.
Shortness of breath (dyspnea): Especially during physical activity or when lying down (orthopnea).
Swelling in the legs and abdomen (edema): Due to fluid buildup.
Palpitations: Feeling of rapid or irregular heartbeats.
Chest pain or discomfort: Caused by reduced blood flow.
Cough: Sometimes with pink, frothy sputum in advanced cases.
Weight loss: Unintentional weight loss due to the chronic nature of the condition.
Ascites: Accumulation of fluid in the abdomen.
Clubbing of fingers: Rare, but can occur in some advanced cases.

The symptoms of EMF are related to its effects on heart function, particularly in the right ventricle or left ventricle, leading to restrictive cardiomyopathy. If left untreated, it can lead to severe heart failure.

What are the causes of endomyocardial fibrosis?

The exact causes of endomyocardial fibrosis (EMF) are not fully understood, but several factors have been associated with its development. These include:

Infections: Chronic parasitic infections like helminthiasis and schistosomiasis have been linked to EMF, particularly in tropical regions.
Autoimmune responses: Some researchers believe that an abnormal immune response could lead to inflammation and fibrosis of the heart lining.
Genetics: There may be a genetic predisposition in certain populations, as EMF is more common in certain regions and families.
Nutritional deficiencies: A diet low in certain nutrients, particularly protein and magnesium, has been suggested as a potential risk factor in endemic areas.
Hypereosinophilia: High levels of eosinophils (a type of white blood cell) in the blood can lead to damage in the heart lining, causing fibrosis. This condition, known as Löffler’s endocarditis, can progress to EMF.
Environmental factors: Living in tropical and subtropical regions, where EMF is more common, may play a role due to a combination of infectious agents, dietary factors, and other environmental influences.
Repeated heart injury: Chronic inflammation or trauma to the heart’s endocardium might result in scarring and fibrosis over time.

EMF is most frequently seen in tropical and subtropical regions, especially in parts of Africa, Asia, and South America, making it more prevalent in populations exposed to these risk factors.

What is the treatment for endomyocardial fibrosis?

Treatment for endomyocardial fibrosis (EMF) focuses on managing symptoms, improving heart function, and preventing complications. Since the condition is progressive and can lead to heart failure, treatment often involves a combination of medications, lifestyle adjustments, and, in some cases, surgery. Common approaches include:

1. Medications

Diuretics: To reduce fluid buildup in the body, helping with swelling (edema) and shortness of breath.
ACE inhibitors or ARBs: These medications help relax blood vessels, improve blood flow, and reduce the strain on the heart.
Beta-blockers: To slow the heart rate, reduce palpitations, and improve heart function.
Anticoagulants: To prevent blood clots, especially if there is an increased risk of thrombosis due to fibrosis.
Corticosteroids or immunosuppressants: If an autoimmune or inflammatory process is suspected, these medications may help reduce inflammation.

2. Surgery

Endocardial resection: In advanced cases, surgery may be required to remove the fibrotic tissue from the heart’s inner lining. This procedure can help improve the heart’s ability to pump blood and relieve symptoms of heart failure.
Valve repair or replacement: If EMF has caused damage to the heart valves, particularly the mitral or tricuspid valves, surgery may be needed to repair or replace them.

3. Heart failure management

Lifestyle changes: Patients are often advised to reduce salt intake, maintain a healthy diet, and avoid strenuous physical activities that could worsen heart function.
Fluid management: Careful monitoring of fluid intake and weight to manage symptoms of heart failure.

4. Treatment of underlying causes

Treatment of infections: If EMF is associated with parasitic infections (e.g., helminths or schistosomiasis), appropriate anti-parasitic medications may be prescribed.
Management of hypereosinophilia: If high eosinophil levels are present, corticosteroids or other therapies to reduce eosinophils may help control the condition.

5. Monitoring and follow-up

Regular check-ups: Patients need ongoing monitoring to assess heart function, detect worsening of fibrosis, and adjust treatments as needed.

In advanced or severe cases, heart transplantation may be considered, though it is rarely an option due to the complexity of the condition.