Republished: recent advances in the epidemiology, diagnosis and treatment of endomyocardial fibrosis in Africa.

Endomyocardial fibrosis (EMF) continues to be an important and disabling disease in many parts of Africa, although its prevalence has declined in some parts of the continent. Increased access to medical care in general and increased availability of echocardiography in some parts of the continent have led to recognition of the disease in areas in which the disease had not been previously reported, and this has given new insights into its natural history. However, the early manifestations of EMF continue to elude clinicians and researchers, and no progress has been made in defining its aetiology. Advances have, however, been made in establishing the epidemiology and improving clinical diagnosis and management, through modern medical therapy and improved surgical techniques. Research is still required to define clinical, biological and echocardiographic markers of early stages of EMF, so that advances in the knowledge of its pathogenesis and pathophysiology can be made. This will hopefully determine preventive measures and avoid the burden of this debilitating condition in this continent.

Schistosomiasis-associated pulmonary hypertension.

Schistosomiasis, a parasite-borne disease, is highly prevalent in Africa and Asia; it is estimated that close to 20 million people worldwide have a severe form of the disease. The chronic form can affect the gastrointestinal system and lead to hepatosplenic disease, and it may cause cardiopulmonary complications, including pulmonary hypertension. The exact pathogenesis of schistosomiasis-associated pulmonary hypertension (Sch-PH) remains unclear, although several mechanisms, including parasitic arterial embolization, pulmonary arteriopathy, and portopulmonary hypertension-like pathophysiology, have been suggested. The immunopathology of the disease is also unclear, although there are similarities with the immunology of idiopathic pulmonary arterial hypertension (PAH). Finally, the treatment of Sch-PH has not been well studied. There is some evidence on treating the underlying infection, with unclear effect on Sch-PH, and advanced PAH therapies are now being suggested, but more studies are needed to confirm their efficacy.

Recent advances in the epidemiology, diagnosis and treatment of endomyocardial fibrosis in Africa.

Endomyocardial fibrosis (EMF) continues to be an important and disabling disease in many parts of Africa, although its prevalence has declined in some parts of the continent. Increased access to medical care in general and increased availability of echocardiography in some parts of the continent have led to recognition of the disease in areas in which the disease had not been previously reported, and this has given new insights into its natural history. However, the early manifestations of EMF continue to elude clinicians and researchers, and no progress has been made in defining its aetiology. Advances have, however, been made in establishing the epidemiology and improving clinical diagnosis and management, through modern medical therapy and improved surgical techniques. Research is still required to define clinical, biological and echocardiographic markers of early stages of EMF, so that advances in the knowledge of its pathogenesis and pathophysiology can be made. This will hopefully determine preventive measures and avoid the burden of this debilitating condition in this continent.

Right ventricular endomyocardial fibrosis.

Endomyocardial fibrosis (EMF) is a neglected tropical cardiomyopathy of unknown etiology and pathogenesis that is common in certain tropical areas of Africa, Asia, and South America. It affects predominantly children and young adults. Endocardial fibrosis is the hallmark of this restrictive cardiomyopathy leading to restriction to diastolic filling of the ventricles with severe atrial dilatation. Endomyocardial fibrosis carries a poor prognosis, due to the late presentation of patients. The salient features of this condition are present in the case presented here of a 14-year-old boy who died from progressive heart failure due to right ventricular EMF. These pathological findings of advanced disease highlight the need for early diagnosis and better understanding of the pathogenesis in order to improve prognosis of this debilitating and fatal disease.