Saturday, April 23, 2011

Endomyocardial Fibrosis

Endomyocardial fibrosis (EMF) is a heart condition which is characterized by fibrosis and thickening of the apical endocardium of the right and left ventricles. The clinical manifestations are largely related to the consequences of restrictive ventricular filling, including left and right sided heart failure. Endomyocardial fibrosis is an idiopathic disorder of the tropical and subtropical regions of the world that is characterized by the development of restrictive cardiomyopathy. Endomyocardial fibrosis is an uncommon cause of congestive cardiac failure and the overall long-term prognosis of this disease is generally considered poor.

Endomyocardial fibrosis is a progressive disease of unknown origin, seriously affecting the heart. Its most obvious feature is a gross change in the makeup of the endocardium (the lining of the heart cavities) of one or both of the the ventricles (lower heart chambers), leading to the replacement of normal cells with fibrous tissue (fibrosis). This process is progressive and leads to the narrowing (constriction) of the right or left ventricular cavities. It may involve the valves between the chambers of the heart as well as the tendon-like cords that fix the valves to the ventricles (chordae tendineae).  

In India, a case of endomyocardial fibrosis that was initially treated by endocardial resection and mitral valve replacement, which was regularly followed-up. The patient underwent successful repair of a paravalvular leak after a period of 17 years. The treatment of left ventricular endomyocardial fibrosis with associated mitral valve disease by endocardial resection and mitral valve replacement is a good surgical option that provides good long-term results.