Article

Endomyocardial Fibrosis: Still a Mystery after 60 Years

Division of Social Medicine and Health Inequalities, Brigham and Women's Hospital, Boston, Massachusetts, USA.
PLoS Neglected Tropical Diseases (Impact Factor: 4.49). 02/2008; 2(2):e97. DOI: 10.1371/journal.pntd.0000097
Source: PubMed

ABSTRACT The pathologist Jack N. P. Davies identified endomyocardial fibrosis in Uganda in 1947. Since that time, reports of this restrictive cardiomyopathy have come from other parts of tropical Africa, South Asia, and South America. In Kampala, the disease accounts for 20% of heart disease patients referred for echocardiography. We conducted a systematic review of research on the epidemiology and etiology of endomyocardial fibrosis. We relied primarily on articles in the MEDLINE database with either "endomyocardial fibrosis" or "endomyocardial sclerosis" in the title. The volume of publications on endomyocardial fibrosis has declined since the 1980s. Despite several hypotheses regarding cause, no account of the etiology of this disease has yet fully explained its unique geographical distribution.

1 Follower
 · 
130 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Endomyocardial fibrosis is a restrictive cardiomyopathy caused by the deposit of fibrotic tissue in the subendocardium. The etiology remains unknown. It can affect one or both ventricles, alter the papillary muscles and produce insufficiency of the auriculoventricular valve of the corresponding ventricle. It is endemic in the tropical countries of Africa, but can also be found in Europe and countries with mild temperatures such as Brazil, Colombia and India. Diagnosis is made by typical clinical, echocardiographic and angiographic findings. Once symptoms are established, the treatment is eminently surgical. This article describes a case of biventricular endomyocardial fibrosis in a malnourished patient with an advanced stage of the disease.
    Revista Colombiana de Cardiologia 11/2014; 21(6):414-418. DOI:10.1016/j.rccar.2014.07.003
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Background: Endomyocardial fibrosis (EMF) is the most common form of restrictive cardiomyopathy worldwide. It has been linked to poverty and various environmental factors, but-for unknown reasons-only some people who live in similar conditions develop the disease. EMF cases cluster within both families and ethnic groups, suggesting a role for a genetic factor in host susceptibility. The human leukocyte antigen (HLA) system is associated with predisposition to various diseases. This two-center study was designed to investigate variation in the HLA system between EMF patients and unaffected controls. We provide the first genetic investigation of patients with EMF, as well as a comprehensive review of the literature. Methods: HLA class I (HLA-A, -B, -C) and class II (DRB1, DQB1) types were determined in 71 patients with severe EMF and 137 controls from Uganda and Mozambique. Chi Square analysis was used to identify any significant difference in frequency of class I and class II HLA types between cases and controls. Results: Compared to ethnically matched controls, HLA-B*58 occurred more frequently in Mozambique patients with EMF and HLA-A*02:02 occurred more frequently in Ugandan patients with EMF. Conclusions: Ample subjective evidence in the historical literature suggests the importance of a genetically susceptible host in EMF development. In this first formal genetic study, we found HLA alleles associated with cases of EMF in two populations from sub-Saharan Africa, with EMF patients being more likely than controls to have the HLA-B*58 allele in Mozambique (p-0.03) and the HLA-A*02:02 in Uganda (p = 0.005). Further investigations are needed to more fully understand the role of genetics in EMF development.
    12/2014; 2014(4):473-481. DOI:10.5339/gcsp.2014.60
  • [Show abstract] [Hide abstract]
    ABSTRACT: Endomyocardial fibrosis (EMF) is a form of restrictive cardiomyopathy common in the tropics and subtropics. The aetiology of EMF is unknown but helminth infestations such as schistosomiasis have been implicated. Two boys aged 8 and 10 years with EMF associated with Schistosoma haematobium, are described. The schistosomes in both cases may have been acquired from contact with contaminated water collected and stored in containers and subsequently used for bathing. Both patients were managed conservatively. Overall prognosis of EMF is poor and this report emphasizes the importance of public health interventions in the control of schistosomiasis.
    Ghana medical journal 12/2014; 48(4):225-7. DOI:10.4314/gmj.v48i4.10

Full-text (3 Sources)

Download
5 Downloads
Available from
Dec 11, 2014