Endomyocardial Fibrosis - Causes, Symptoms, Treatment

A Complete Guide to Endomyocardial Fibrosis and Its Causes

Heart health is a top priority for keeping the body running, just like an engine powers a car. For people with hypereosinophilic syndrome (HES), endomyocardial fibrosis (EMF) is a persistent risk to their health. It is a rare heart condition where scar tissues occur on the endocardium of the heart.

Let us understand the complex condition by shedding light on causes, symptoms, age of occurrence, diagnosis and treatment.

What is Endomyocardial Fibrosis Hypereosinophilic Syndrome?

Initially, in 1936, Loeffler described endomyocardial fibrosis as "fibroplastic parietal endocarditis with blood eosinophilia". He defined EMF as a major characteristic of the cardiovascular abnormality in hypereosinophilic syndrome.

Endomyocardial fibrosis is a mysterious form of restrictive cardiomyopathy that predominantly occurs in subtropical and tropical regions. This causes scar tissue in the inner lining of the heart, which builds up to functional issues in relaxing and filling with blood. This disease affects the left and right ventricles, causing dyspnoea and fatigue. Endomyocardial fibrosis often causes several complications, including:

• Arrhythmias
• Heart Failure
• Thromboembolic, which increases the risk of stroke

What is the Cause of Myocardial Fibrosis?

Precise causes of the conditions are still not clear, but infection and environmental factors can significantly trigger fibrosis and inflammation of the endocardium. Some potential causes include:

• Infectious and Environmental Factors: Parasitic infections, specifically schistosomiasis, are the main cause of EMF. It develops because the parasite triggers an inflammatory response in the heart, which causes fibrotic changes.

Excessive exposure to environmental pollutants or toxins plays a role in the process of developing endomyocardial fibrosis.

• Nutritional Deficiency: Nutritional deficiencies (e.g., of magnesium) are considered a potential contributing environmental factor, but this is not a cause of HES-related heart disease..
• Autoimmune Mechanism: In Hypereosinophilic Syndrome (HES), the heart damage (Loeffler's endocarditis) is caused by toxic substances released by the excessive number of eosinophils, which lead to inflammation and scarring. This is not primarily an autoimmune process. 
• Lifestyle and Socioeconomic Factors: Poor living places or conditions and inadequate clinical care services can increase the risk of causing endomyocardial fibrosis. In addition, people with lower socioeconomic status face problems regarding higher prevalence of infections, poor nutrition, and limited healthcare access. Hence, socioeconomic status is a higher risk of EMF.

At What Age Does Endomyocardial Fibrosis Occur?

Endomyocardial fibrosis typically affects individuals from lower socioeconomic backgrounds and is most prevalent in older children and young adults, particularly between the ages of 5 and 15. It may also appear in the elderly, especially those over 60 or 70 years old. However, it is rarely seen in children under the age of 4, making early childhood an uncommon period for its onset.

What are the common symptoms of endomyocardial fibrosis?

The common symptoms of endomyocardial fibrosis are similar to the signs of heart failure, including:

• Oedema
• Fluid builds up in the abdomen.
• Visible swelling of the jugular vein in the neck area
• Breathing difficulties and shortness
• Weakness and fatigue
   

Other symptoms that may vary depending on the condition:

• Palpitations
• Loss of consciousness
• Blood clotting in the heart
• Weight loss
• Purple or dark red coloured streaks under fingernails
• Chest pain
   

What are the treatment options for endomyocardial fibrosis?

Endomyocardial fibrosis requires a combination of blood tests, clinical assessments, and imaging tests, such as cardiac MRI and echocardiography, for diagnosis. The laboratory tests visualise heart function, the presence of fibrosis, and infiltration. In addition, the treatment of EMF involves medical care and surgical interventions.

Surgical Treatment Options

Surgical treatment deals with severe symptoms when other options have failed to treat the disease.

• Endocardial Decortication: A definitive process of taking out the fibrous endomyocardium to manage obstructions and improve blood flow in the heart.
• Valve Replacement or Repair: This process repairs or replaces the tricuspid or mitral valve to restore function and affect the survival rate favourably.
• Cavopulmonary Shunting: A process of diverting blood to the lungs and reducing the functional load on the right ventricle.
• Heart Transplantation: This surgical option is considered for advanced heart failure. It improves cardiac output and relieves pulmonary congestion and oedema.
   

Medical Care Options

Medical care primarily treats symptoms and future complications of EMF:
 

• Diuretics: This medicine reduces fluid buildup and improves blood flow and heart function. It manages heart failure-related problems, such as leg swelling and shortness of breath.
• Beta-blockers: These confer benefits to patients by reducing the workload on the heart in controlling heart rate.
• ACE Inhibitors: Angiotensin-converting enzyme inhibitors reduce blood pressure, which improves heart function and minimises the disease progression.
• Anticoagulation: In case of suspected thrombosis and atrial fibrillation, anticoagulation prevents blood clots and further health issues.
   

Endomyocardial fibrosis is a condition of restrictive cardiomyopathy in which the inner lining of the heart becomes scarred and thickened, causing heart failure. This disease can lead to major health complications, such as valvular dysfunction, pulmonary hypertension, and sudden death.

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