Endomyocardial fibrosis is a rare heart condition characterized by abnormal thickening and scarring of the heart’s inner lining. This progressive disease primarily affects the heart’s pumping chambers, impairing their function. While uncommon in Western countries, it is a significant health challenge in tropical and subtropical regions. The condition can severely impact heart health, often leading to heart failure.
What is Endomyocardial Fibrosis?
Endomyocardial fibrosis (EMF) involves fibrous tissue formation within the endocardium, the thin membrane lining the heart’s ventricles. This scarring typically occurs in the ventricles, specifically at the apex and along the inflow tracts. Fibrous deposits can become quite thick, sometimes exceeding 1 centimeter, and may extend into the heart muscle.
This scarring makes ventricular walls stiff and less flexible, restricting the heart’s ability to relax and fill with blood. As a result, the heart struggles to pump enough blood, leading to restrictive cardiomyopathy. Fibrotic changes can also affect heart valves, particularly the mitral and tricuspid valves, potentially causing them to leak.
Recognizing the Signs
Symptoms of endomyocardial fibrosis vary depending on affected heart parts and disease advancement. Early symptoms can be general and non-specific, making diagnosis challenging. As the disease progresses and heart function declines, more noticeable signs emerge.
Individuals may experience persistent fatigue and shortness of breath, particularly during physical activity or when lying flat. This occurs because the heart cannot effectively circulate oxygenated blood. Fluid retention is common, manifesting as swelling in the legs, ankles, and abdomen. This fluid buildup is a direct consequence of the heart’s reduced pumping efficiency. Some individuals might also notice irregular heartbeats or palpitations.
Understanding the Causes
The precise cause of endomyocardial fibrosis often remains unclear, classifying it as idiopathic. However, research suggests environmental, infectious, and immunological factors may contribute to its development. The condition is notably prevalent in tropical and subtropical areas of Africa, Asia, and South America.
One hypothesis links EMF to parasitic infections, such as the Loa loa filaria worm, though its exact role is still under investigation. Immunological responses, possibly triggered by infections or other environmental exposures, are also considered contributors. Eosinophil involvement has been observed in some cases, suggesting a possible hypersensitivity reaction.
Diagnosis and Management Approaches
Diagnosing endomyocardial fibrosis involves a comprehensive assessment. A physical examination may reveal signs of heart failure, such as murmurs or fluid accumulation. Imaging tests are crucial for confirming diagnosis and assessing heart damage. An echocardiogram, which uses sound waves to create images of the heart, is particularly important. This test can reveal characteristic endocardial thickening, restricted ventricular filling, and potential valve abnormalities.
Other diagnostic tools include an electrocardiogram (ECG) for electrical abnormalities, a chest X-ray to evaluate heart size and lung congestion, and cardiac magnetic resonance imaging (MRI) for detailed views of heart structure and fibrosis. These tests help clinicians understand the specific patterns of fibrosis and guide treatment decisions.
Management approaches aim to alleviate symptoms, prevent complications, and improve heart function. Medical management often involves medications for heart failure. Diuretics are commonly prescribed to reduce fluid buildup and swelling. Medications to control heart rate and rhythm may be used to manage arrhythmias. Anticoagulants are often necessary to prevent blood clots, a significant risk due to stagnant blood flow in fibrotic chambers.
For more advanced cases, surgical intervention may be considered. Endocardiectomy, a procedure to remove thickened, fibrotic tissue from the heart’s inner lining, can help restore ventricular function. Repair or replacement of affected heart valves may also be performed to improve blood flow. While surgery can offer significant improvement, the decision depends on the individual’s overall health and the disease’s severity.
Living with Endomyocardial Fibrosis
Living with endomyocardial fibrosis requires ongoing medical care focused on managing symptoms and preventing complications. The long-term outlook varies, influenced by the disease stage at diagnosis and treatment effectiveness. Early diagnosis and intervention can improve outcomes.
Potential complications include severe heart failure, irregular heart rhythms (arrhythmias), and blood clots that can lead to strokes or pulmonary embolisms. Regular follow-up with a cardiologist is important to monitor heart function and adjust treatments. Lifestyle adjustments, such as dietary modifications for fluid and sodium intake, may also be recommended. Patient support and education are valuable for coping with this condition.