Limits...
Endomyocardial fibrosis: still a mystery after 60 years.

Bukhman G, Ziegler J, Parry E - PLoS Negl Trop Dis (2008)

Bottom Line: The pathologist Jack N.Since that time, reports of this restrictive cardiomyopathy have come from other parts of tropical Africa, South Asia, and South America.Despite several hypotheses regarding cause, no account of the etiology of this disease has yet fully explained its unique geographical distribution.

View Article: PubMed Central - PubMed

Affiliation: Division of Social Medicine and Health Inequalities, Brigham and Women's Hospital, Boston, Massachusetts, USA. gbukhman@partners.org

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.

Show MeSH

Related in: MedlinePlus

Top, echocardiogram in a 25-y-old man with predominantly right ventricular EMF from eastern Rwanda.Apical four-chamber view. Note the marked dilatation of the right atrium. RV = right ventricle, RA = right atrium, LV = left ventricle, LA = left atrium. Bottom, massive ascites in the same patient.
© Copyright Policy
Related In: Results  -  Collection


getmorefigures.php?uid=PMC2254164&req=5

pntd-0000097-g001: Top, echocardiogram in a 25-y-old man with predominantly right ventricular EMF from eastern Rwanda.Apical four-chamber view. Note the marked dilatation of the right atrium. RV = right ventricle, RA = right atrium, LV = left ventricle, LA = left atrium. Bottom, massive ascites in the same patient.

Mentions: Subendocardial fibrosis of the apices and inflow tracts of the right ventricle, left ventricle, or both defines the disease [7],[8]. This restrictive scarring prevents ventricular filling, and tethering of the papillary muscles leads to valvular regurgitation (Figure 1; Video S1). A review of autopsies in Uganda between 1959 and 1969 emphasized the poor prognosis of this condition, with an average survival of 2 y after symptom onset [9]. Later series from Brazil and India found more variability in the course of medically treated patients and echoed findings from southern Nigeria of both acute and chronic forms of the disease [10],[11],[12]. The advent of surgical resection and valvular replacement during the 1970s promised 10-y survival rates as high as 68% for selected patients, but at the price of high peri-operative mortality [13],[14],[15],[16],[17]. Unfortunately, EMF has most affected those regions least equipped with cardiovascular surgery.


Endomyocardial fibrosis: still a mystery after 60 years.

Bukhman G, Ziegler J, Parry E - PLoS Negl Trop Dis (2008)

Top, echocardiogram in a 25-y-old man with predominantly right ventricular EMF from eastern Rwanda.Apical four-chamber view. Note the marked dilatation of the right atrium. RV = right ventricle, RA = right atrium, LV = left ventricle, LA = left atrium. Bottom, massive ascites in the same patient.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2254164&req=5

pntd-0000097-g001: Top, echocardiogram in a 25-y-old man with predominantly right ventricular EMF from eastern Rwanda.Apical four-chamber view. Note the marked dilatation of the right atrium. RV = right ventricle, RA = right atrium, LV = left ventricle, LA = left atrium. Bottom, massive ascites in the same patient.
Mentions: Subendocardial fibrosis of the apices and inflow tracts of the right ventricle, left ventricle, or both defines the disease [7],[8]. This restrictive scarring prevents ventricular filling, and tethering of the papillary muscles leads to valvular regurgitation (Figure 1; Video S1). A review of autopsies in Uganda between 1959 and 1969 emphasized the poor prognosis of this condition, with an average survival of 2 y after symptom onset [9]. Later series from Brazil and India found more variability in the course of medically treated patients and echoed findings from southern Nigeria of both acute and chronic forms of the disease [10],[11],[12]. The advent of surgical resection and valvular replacement during the 1970s promised 10-y survival rates as high as 68% for selected patients, but at the price of high peri-operative mortality [13],[14],[15],[16],[17]. Unfortunately, EMF has most affected those regions least equipped with cardiovascular surgery.

Bottom Line: The pathologist Jack N.Since that time, reports of this restrictive cardiomyopathy have come from other parts of tropical Africa, South Asia, and South America.Despite several hypotheses regarding cause, no account of the etiology of this disease has yet fully explained its unique geographical distribution.

View Article: PubMed Central - PubMed

Affiliation: Division of Social Medicine and Health Inequalities, Brigham and Women's Hospital, Boston, Massachusetts, USA. gbukhman@partners.org

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.

Show MeSH
Related in: MedlinePlus