Latest Research on Endomyocardial Fibrosis: Dec 2020

Endomyocardial Fibrosis: Still a Mystery after 60 Years

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]

Löffler’s endocarditis and Davies’ endomyocardial fibrosis

This paper reports a review of the literature on 90 cases of endomyocardial lesions associated with eosinophilia, and a personal study of the histology of 30 cases, comparing them with 32 other fibrotic lesions including E.M.F. from Uganda, Nigeria, and Brazil. It is argued that Löffler’s endocarditis is a specific disease entity with a pathologic spectrum ranging from acute myocardial inflammation to hyaline fibrous endocardial thickening. The comparative study showed that Löffler’s endocarditis (in the fibrotic stage) could not be distinguished histologically from Davies’ E.M.F. This provides further support for the identity of the two diseases. [2]

Clinical course of endomyocardial fibrosis.

The survival pattern, morbidity, and clinical course of 145 patients with endomyocardial fibrosis who were followed up between November 1975 and June 1987 were studied. The diagnosis was confirmed in all cases by cardiac angiography, or echocardiography, or necropsy. Percentage survival at the end of one and 9.5 years was 76.11 and 26.35 respectively. History, physical examination, electrocardiography, and cardiac catheterisation were studied at the first presentation. The determinants of early mortality were studied by univariate Kaplan-Meier estimates compared by the log rank test and Cox proportional hazards multiple regression analysis. Significant univariate predictors of early mortality were QRS axis above +90 degrees, intraventricular conduction delay (QRS duration greater than 0.12 s), duration of symptoms before presentation, New York Heart Association functional classes III and IV, presence of embolic episodes, right atrial mean pressures greater than 20 mm Hg, right ventricular end diastolic pressure greater than 20 mm Hg, and aortic oxygen saturation less than 85%. The significant multivariate predictors of mortality were cyanosis, New York Heart Association functional class at first presentation, and right atrial mean pressure greater than 20 mm Hg. The bleak prognosis of endomyocardial fibrosis did not substantially improve despite advances in the medical management of congestive cardiac failure during the period of the study. [3]

Increasing Role of Magnetic Resonance in Diagnosis and Prognosis of Arrhythmogenic Right Ventricle Dysplasia/Cardiomyopathy

A 33-year-old woman with previous diagnosis of asymptomatic frequent premature ventricular complexes (PVC), without documented structural disease in echocardiography. She presented recently with palpitations and dizziness. Another echocardiogram was performed suggesting for the first time right ventricle (RV) dilation and possible hypokinesia of RV apex. Electrocardiogram showed T-wave inversion in V1-V2 and Epsilon wave in V1. Holter documented 2869 polymorphic isolated PVC. Cardiovascular magnetic resonance (CMR) showed moderate RV dilation (end-diastolic volume of 125 ml/m2), RV systolic dysfunction (RV ejection fraction 33%) with RV free wall hypokinesia, a focal area of dyskinesia (“buldging”) in RV outflow tract and trabecular disarray. There was no evidence of intra-myocardial fat. Positive Late gadolinium enhancement was evident in RV free wall and RV outflow tract. [4]

Death Due to Low Voltage Electric Shock Induced Myocarditis

Low voltage electric shock resulting in myocarditis induced delayed death is a rarity and has not been reported so far, to the best of our knowledge. The definitive diagnosis is autopsy based as it has variable clinical presentations. We report such a case where in the histopathologic findings of myocarditis came as a surprise during microscopic evaluation of the autopsy sections in a case with an apparently normal heart on gross examination. The present case mandates a careful microscopic examination of autopsy sections in cases of electrocution. [5]

Reference

[1] Bukhman, G., Ziegler, J. and Parry, E., 2008. Endomyocardial fibrosis: still a mystery after 60 years. PLoS Negl Trop Dis, 2(2), p.e97.

[2] Brockington, I.F. and Olsen, E.G., 1973. Löffler’s endocarditis and Davies’ endomyocardial fibrosis. American Heart Journal, 85(3), pp.308-322.

[3] Gupta, P.N., Valiathan, M.S., Balakrishnan, K.G., Kartha, C.C. and Ghosh, M.K., 1989. Clinical course of endomyocardial fibrosis. Heart, 62(6), pp.450-454.

[4] Rosa, S. A., Castela, S., Matos, P., da Silva, M. N. and Tavares, N. J. (2016) “Increasing Role of Magnetic Resonance in Diagnosis and Prognosis of Arrhythmogenic Right Ventricle Dysplasia/Cardiomyopathy”, Journal of Advances in Medicine and Medical Research, 18(4), pp. 1-8. doi: 10.9734/BJMMR/2016/28394.

[5] Kundu, R., Singh Punia, R. and Harish, D. (2018) “Death Due to Low Voltage Electric Shock Induced Myocarditis”, Cardiology and Angiology: An International Journal, 7(2), pp. 1-4. doi: 10.9734/CA/2018/39585.

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