From the Guidelines
Endomyocardial fibrosis (EMF) should be managed with a focus on supportive care, including diuretics, ACE inhibitors, and beta-blockers to reduce symptoms and improve cardiac function, as recommended by the American Heart Association 1. The primary goal of treatment is to alleviate symptoms of heart failure, such as fluid overload, and to improve quality of life.
- Diuretics like furosemide (20-80 mg daily) or spironolactone (25-100 mg daily) are first-line treatments to reduce fluid overload.
- ACE inhibitors such as enalapril (2.5-20 mg twice daily) or beta-blockers like metoprolol (12.5-200 mg daily) may help improve cardiac function.
- Anticoagulation with warfarin (target INR 2-3) is recommended for patients with atrial fibrillation or ventricular thrombi to prevent embolic events, as suggested by the American Heart Association 1. Advanced cases may require surgical intervention, including endocardiectomy to remove fibrotic tissue or valve replacement if significant valvular regurgitation is present.
- Heart transplantation is considered in end-stage disease. The pathophysiology of EMF involves fibrosis of the endocardium and subendocardial layers, leading to ventricular stiffness, impaired filling, and ultimately restrictive physiology, as described in the American Heart Association statement 1. This condition is more prevalent in tropical regions and may be associated with eosinophilia, parasitic infections, or nutritional factors, though the exact etiology remains unclear. Endomyocardial biopsy may be considered in certain clinical scenarios, such as unexplained restrictive cardiomyopathy, as recommended by the American Heart Association, the American College of Cardiology, and the European Society of Cardiology 1.
From the Research
Definition and Characteristics of Endomyocardial Fibrosis
- Endomyocardial fibrosis (EMF) is a cause of restrictive cardiomyopathy with an unclear aetiopathogenesis, characterized by ventricular fibrosis that causes diastolic dysfunction and atrioventricular regurgitation 2.
- The disease is predominant in tropical and subtropical regions of the developing world, and is often found in children and adolescents 2, 3.
- EMF is characterized by fibrotic thickening of the endocardium and myocardium of one or both ventricles, leading to impaired filling and valvular insufficiency 3.
Epidemiology and Prognosis
- The epidemiological trends of EMF are difficult to ascertain, but it is known to be an important cause of heart failure in endemic areas, accounting for up to 20% of cases in some regions 3.
- The prognosis of EMF is poor, with untreated patients having a very poor prognosis, and medical management only able to mitigate symptoms for a time without providing a curative benefit 4.
- Surgery can increase survival, especially in patients with advanced heart failure, but medical care remains challenging, with one-third to half of patients with advanced disease dying within 2 years 3.
Treatment and Management
- Surgical treatment, including endocardial decortication and atrioventricular valve replacement, can improve survival and symptoms in patients with EMF 5, 6.
- The surgical procedure is considered palliative, with possible limitations including the need for a valve prosthesis, cardiac conduction disturbances, and the possibility of recurrence of the endocardial fibrosis 5.
- Medical management can mitigate symptoms, but has no curative benefit, and increased awareness, advocacy, and research are needed to further understand and improve treatment of EMF 4.
Research and Future Directions
- A major focus of research is the identification of biomarkers of preclinical disease and new therapeutic targets for EMF 2.
- Collaborative multidisciplinary research and cross-learning from other fibrotic conditions may help improve survival rates and quality of life for patients with EMF 2.
- Further studies are needed to understand the etiology and pathogenesis of EMF, and to develop effective treatments for this neglected tropical cardiomyopathy 4, 3.