Endomyocardial fibrosis: Clinical profile and role of surgery in management

Cherian, K.M.; John, Thomas; Abraham, K A

doi:10.1016/0002-8703(83)90509-4

Cited by 14 publications

(5 citation statements)

References 7 publications

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“…In the absence of surgical intervention, the mean period of survival after the onset of symptoms is two years. 22 A similar cardiac disease has been described in patients with eosinophilic leukemia and in those with Löffler's syndrome, 23 but the prolonged course, the absence of apical obliteration on echocardiographic and angiographic studies, and the absence of eosinophilia in this case make these disorders very unlikely.…”

supporting

confidence: 61%

Case 34-1996

McGovern¹,

Flieder²

1996

N Engl J Med

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A 50-year-old woman was admitted to the hospital because of cardiac arrest in ventricular fibrillation.She had been in excellent health until four years earlier, when she abruptly lost consciousness. Evaluation elsewhere revealed complete heart block. An electronic cardiac pacemaker, in DDD mode, was implanted, with symptomatic improvement.Three and a half years before admission, the patient had exertional dyspnea and tachycardia. Disopyramide was administered, but ventricular fibrillation developed within a week; the drug was discontinued. Cardiac ultrasonography showed a dilated left ventricle, with akinesis of the anterior wall, the middleto-distal portion of the septum, and the cardiac apex. The left ventricular ejection fraction was 25 to 30 percent. Cardiac catheterization revealed borderline dilatation of the left ventricle, with hypokinesis of the anterior and inferior walls, apical akinesis, and a left ventricular ejection fraction of 59 percent after a premature ventricular contraction. The coronary arteries appeared normal. Enalapril, a diuretic, and digoxin were prescribed, with improvement in the patient's condition. Cardiac ultrasonography performed eight months before admission showed diffuse hypokinesis of the left ventricle, which was of normal size. The left atrial diameter was 50 mm. The left ventricular ejection fraction was 55 percent.Four months before admission, exertional dyspnea recurred, with atypical chest pain. Another cardiac ultrasonographic examination showed mild mitral regurgitation, a left atrial diameter of 39 mm, and mild, concentric hypertrophy of the left ventricle, which was normal in size and systolic function. The right ventricle appeared normal. An exercise tolerance test showed an increase in the heart rate from 92 at base line to a peak value of 105; the blood The New England Journal of Medicine Downloaded from nejm.org at EMORY UNIVERSITY on August 11, 2015. For personal use only. No other uses without permission. Volume 335 Number 18 ؒ 1379 *Measurements were made while the patient was receiving 100 percent oxygen. †Measurements were made while the patient was breathing an enriched oxygen mixture. The New England Journal of Medicine Downloaded from nejm.org at EMORY UNIVERSITY on August 11, 2015. For personal use only. No other uses without permission.

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supporting

confidence: 61%

Case 34-1996

McGovern¹,

Flieder²

1996

N Engl J Med

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“…7,13 In sub-Saharan Africa, EMF has been predominantly clustered in Uganda, along the low-lying coastal belt of Mozambique, and in some part of West Africa, but sporadic cases have also been reported in Congo and Malawi, among others. [14][15][16][17][18][19][20][21][22][23][24] India has witnessed a striking burden of EMF along the coastal area and rain forest of Kerala State, [25][26][27] and in China, a high prevalence has been found in the province of Guangxi. 28 In South America, EMF has been reported mainly in Brazil and Colombia.…”

Section: Epidemiologymentioning

confidence: 99%

Tropical Endomyocardial Fibrosis

et al. 2016

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Abstract-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.

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“…(69) It should be performed before irreversible cardiac and hepatic damage occurs. (70) In Africa due to the lack of human and material resources for open-heart surgery in most endemic areas, the surgical experience has been growing slowly.…”

Section: Managementmentioning

confidence: 99%

Neglected cardiomyopathies in Africa

Mocumbi

2017

SAHJ

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Size of the problemCardiomyopathies constitute a huge burden to both communities and health systems in Africa. Together with rheumatic heart disease, tuberculous pericarditis, systemic hypertension and its complications, cardiomyopathies are the major causes of heart disease in black African populations, causing great morbidity and mortality in young and economically active people (6,7) and being responsible for more than 20% of all admissions for cardiovascular diseases in both children and adults.EMF, the most common cardiomyopathy in African children and adolescents, shows great geographic variation with areas of higher incidence being the tropical regions of East, Central and West of the continent, (9) where it accounts for up to 20% of cases of heart failure. (5,7,10)

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Endomyocardial fibrosis: Clinical profile and role of surgery in management

Cited by 14 publications

References 7 publications

Case 34-1996

Case 34-1996

Tropical Endomyocardial Fibrosis

Neglected cardiomyopathies in Africa

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