Endomyocardial fibrosis: Seven decades later in the Nigerian setting

Abstract

Background: Endomyocardial fibrosis (EMF), an obliterative cardiomyopathy of uncertain etiology, is the most common restrictive cardiomyopathy worldwide with no specific treatment and usually associated with poor prognosis due to late presentation. First described in the literature in 1946 among Africans, the prevalence seem to be decreasing, but without much change in its management in the region due to late presentation and inadequate surgical responsiveness. Earlier literature described EMF as a disease of the rain forest, only restricted to dwellers in the coastal regions. Despite the growing body of knowledge on this condition, there is not enough corresponding advancement in therapeutic interventions for the sufferers in Nigeria. Materials and Methods: Between May 2010 and July 2011, we reviewed cases of endomyocardial fibrosis presenting to the cardiology unit of Jos University Teaching Hospital, Plateau State Nigeria. The institutional board of the hospital approved the review, and consent was obtained from the patients/guardians. Effort was made to withhold identity of the patients in accordance with established protocol. Result: The first case was an 18-year-old female student with features of chronic heart failure who has earlier been misdiagnosed as having rheumatic valvular heart disease, but had biventricular EMF. Case 2 was a 33-year-old man who presented with breathlessness and hemoptysis and was erroneously on treatment for tuberculosis. Echocardiography revealed right ventricular EMF. The final case was a 27-year-old farmer with cough, progressive shortness of breath and abdominal swelling. Holter monitoring revealed ventricular and atrial arrhythmias, and echocardiography showed pericardial effusion with features of right ventricular EMF. He had bouts of arrhythmia with collapse, and finally died suddenly. Conclusion: There have been little advances in management of EMF seven decades after it was discovered, and despite being one of the major causes of heart failure in the tropics, awareness and response to management in these areas have lagged behind.