Löffler's endocarditis and Davies' endomyocardial fibrosis

Background Endomyocardial fibrosis is the most common restrictive cardiomyopathy worldwide. It has no specific treatment and carries a poor prognosis, since most patients present with advanced heart failure. On the basis of clinical series, regional variations in distribution have been reported within several countries in Africa, Asia, and South America, but large-scale data are lacking on the epidemiologic features and early stages of the disease. Methods We used transthoracic echocardiography to determine the prevalence of endomyocardial fibrosis in a rural area of Mozambique. We screened a random sample of 1063 subjects of all age groups selected by clustering. Major and minor diagnostic criteria were defined, and a severity score was developed and applied. Cases were classified according to the distribution and severity of the lesions in the heart. Results The estimated overall prevalence of endomyocardial fibrosis was 19.8%, or 211 of 1063 subjects (95% confidence interval [CI], 17.4 to 22.2). The pr...

https://www.nejm.org/doi/pdf/10.1056/NEJMoa0708629

A Population Study of Endomyocardial Fibrosis in a Rural Area of Mozambique

Relation between eosinophilia and endomyocardial disease

In 19 patients right ventricular infarction was diagnosed on the basis of electrocardiographic features of acute inferior infarction and clinical evidence of elevation of systemic venous pressure and an absence of pulmonary congestion. Right heart catheterization documented elevated right ventricular end-diastolic pressure (mean 15.5 mm Hg) and commensurate right atrial pressure (mean 14.9 mm Hg). In all patients the pulmonary capillary wedge pressure (mean 13.2 mm Hg) was exceeded or equaled by the right ventricular end-diastolic pressure, suggesting a disproportionate reduction in right ventricular compliance or contractile function, or both. Thirteen patients were hypotensive (systolic blood pressure less than 100 mm Hg on admission), including six patients with cardiogenic shock. Right ventricular infarction is an uncommon and potentially reversible cause of cardiogenic shock;yet, in the experimental model, isolated right ventricular damage is relatively well tolerated. To identify the factors associated with systemic hypotension, data from patients with and without compromised systemic hemodynamic function were compared. In hypotensive patients, the right ventricular end-diastolic pressure was significantly higher (16.8 versus 12.8 mm Hg;p There was one hospital death (mortality rate 5.3 percent). Clinical management generally consisted of administration of fluids and digitalis and implantation of a temporary pacemaker. This study emphasizes the relatively favorable prognosis of this condition and suggests that aggressive diagnosis and management are appropriate.

Hemodynamic spectrum of “dominant” right ventricular infarction in 19 patients

Tropical endomyocardial fibrosis (EMF) is a neglected disease of poverty that afflicts rural populations in tropical low-income countries, with some certain high-prevalence areas. Tropical EMF is characterized by the deposition of fibrous tissue in the endomyocardium, leading to restrictive physiology. Since the first descriptions in Uganda in 1948, high-frequency areas for EMF have included Africa, Asia, and South America. Although there is no clear consensus on a unified hypothesis, it seems likely that dietary, environmental, and infectious factors may combine in a susceptible individual to give rise to an inflammatory process leading to endomyocardial damage and scar formation. The natural history of EMF includes an active phase with recurrent flare-ups of inflammation evolving to a chronic phase leading to restrictive heart failure. In the chronic phase, biventricular involvement is the most common presentation, followed by isolated right-sided heart disease. Marked ascites out of proportion to peripheral edema usually develops as a typical feature of EMF. EMF carries a very poor prognosis. In addition to medical management of heart failure, early open heart surgery (endocardectomy and valve repair/replacement) appears to improve outcomes to some extent; however, surgery is technically challenging and not available in most endemic areas. Increased awareness among health workers and policy makers is the need of the hour for the unhindered development of efficient preventive and therapeutic strategies.

Tropical Endomyocardial Fibrosis: Natural History, Challenges, and Perspectives.

Endomyocardial fibrosis is a relatively common form of heart disease in Uganda (Davies, 1948; Shaper and Williams, 1960) and accounts for some 10 per cent of heart disease seen at necropsy in Kampala (Davies, 1961). It is characterized in the established condition by fibrosis in the endocardium and subjacent myocardium affecting particularly the inflow tract and the apex of one or both ventricles. The aetiology of this disorder is not known, and hypotheses have been put forward in attempts to incriminate virus or filarial infections, plantain diets, and rheumatic heart disease. The disorder has also been described in West Africa, Ceylon, South India, and Central Africa, and wellauthenticated cases have been seen in Europeans resident in tropical areas (Brockington, Olsen, and Goodwin, 1967). Mulago Hospital, Kampala, is situated in Buganda, the largest province of Uganda, and about half the patients admitted to the hospital belong to the local Ganda tribe (Fig. 1). There is also a large immigrant population in Buganda, coming in particular from Rwanda and Burundi (herein referred to as 'Rwandans') and from the Western Province of Uganda (Kigezi, Ankole, Toro, and Bunyoro districts). An analysis of the tribal origins of cases of endomyocardial fibrosis coming to necropsy at Mulago Hospital in the period 19501961 showed a preponderance of this condition among those groups immigrant to Buganda, in particular those originating from Rwanda and Burundi. The condition was far less common than expected among the indigenous Ganda people (Shaper and Coles, 1965). This analysis of the tribal origins of subjects with endomyocardial fibrosis has now been extended to cover the period 1950-1965, and a similar analysis

https://heart.bmj.com/content/heartjnl/30/3/391.full.pdf

M. S. R. Hutt

Papers

Necropsy study of endomyocardial fibrosis and rheumatic heart disease in Uganda 1950-1965.