Endomyocardial Fibrosis: Diagnosis and Management

Abstract

Introduction:

Endomyocardial fibrosis (EMF) is a form of restrictive cardiomyopathy of unknown etiology, characterized by endocardial fibrosis of the apices and inflow tracts of the right ventricle, left ventricle or both. The majority of people who suffer high morbidity and mortality are children and young females of the poor and deprived communities of tropical Africa. EMF has been reported from other subtropical countries; Egypt, Nigeria, Brazil, Kerala in India, and Sudan. It is exceedingly rare in Europe and North America; however few cases have been reported from China and Japan. In Uganda, it accounted for 25% of cases who reported for echocardiography and 20% in a random population sample in Mozambique. Although ethnicity, diet, poverty, eosinophilia, infection, and malaria have been shown to be associated with EMF, the etiology of the disease still remains undetermined.

Diagnosis:

Echocardiography is now the gold standard tool for the diagnosis of EMF. There are five prime diagnostic echocardiographic features of EMF; apical fibrosis, ventricular wall fibrosis, huge atrium, atrioventricular valve regurgitation and obliteration of ventricular cavity. The presence of pericardial effusion, endocardium fibrous shelf, and layering of the posterior wall lend further diagnostic support.

Treatment:

Most patients are seen at a late stage of the disease with heart failure, consequently, medical care is directed accordingly and includes diuretics, angiotensin enzyme inhibitors, and beta-blockers. Surgical care includes pericardectomy, a relatively safe procedure that leads to some improvement in heart failure symptoms. However, endocardial decortication seems to be more definitive and beneficial for many patients with advanced disease. Successful surgery has a clear benefit in terms of symptoms and seems to affect survival favorably. The prognosis is poor and the duration of illness from the time of presentation to death was less than one year in 43.5% of patients, three years in 39.1% of patients, and three to twelve years in 17.4% of patients.

Conclusion:

EMF is a mysterious disease whose etiology is still unraveling. The afflicted individuals are mostly young females and children who succumb to high rates of morbidity, and mortality from heart failure. Medical and surgical treatment are both practiced with results varying from temporary relief of symptoms to surgery with high mortality rates.

Keywords:

• endomyocardial fibrosis
• apical fibrosis
• ventricular outflow tract fibrosis
• ascites
• pericardiectomy
• endocardial decortication

Disclosure

The author reports no conflicts of interest in this work.