Tropical endomyocardial fibrosis is a peculiar disease involving the heart. Recent reports on it have appeared in alphabetical order in Brazil, Casablanca, France, India, Mozambique and the United Kingdom. Therefore, this case report from Nigeria is deemed to be worthy of documentation.

Keywords: Heart, Muscle, Fibrosis, Etiology, Early death

CASE REPORT

External appearances: The body was that of an emaciated young man who was very pale. The ankles pitted on pressure. Both legs were slightly sabre shaped, especially on the left, whose convexity was surmounted by a large healed ulcer. The umbilicus was absent, there being a transverse abdominal surgical scar, which suggested having undergone operation probably for the repair of umbilical hernia. The abdomen was greatly distended and a mass was palpable on the left side. The right breast was slightly enlarged. There was no lymphadenopathy or finger clubbing.

Internal appearances:

Chest: Both pleural cavities contained much straw colored exudates, the lungs lying free, except for firm adhesions over the left apex. On section, both lungs were edematous. The pericardium was greatly dilated, occupying much of the chest cavity. It contained about a liter of slightly blood stained fluid. The heart itself was picturesque. A few whitish plaques were noted on the external surfaces of the atria and ventricles, especially the left ventricle. Enlargement was striking on the right side, especially the atrium. Both ventricles exhibited thickened walls. The atrial walls were dilated, being paper thin on the right side. The endocardial surface was glistening white throughout the left atrium, especially around the valves. The right chamber was less impressive in this respect. As for the ventricles, the left was also more generalized and thicker, especially in the apical area, whereas in the right chamber this was limited to the posterior areas. Of the valves, both the pulmonary and the aortic ones were unexceptional, but both atria-ventricular ones were thickened. Their chordae tendinae were shortened, especially on the right side. No thrombi were found.

Head: Not examined. 

Microscopy: The myocardium was the seat of considerable fibrosis which special staining confirmed to be more marked nearer the endocardium. Lymphocytic infiltrates were noted in the epicardium. Both layers of the pericardium were gummed up in parts by inflammatory infiltrates which featured lymphocytes and plasma cells with occasional polymorphs. The peritoneum was similarly affected, fibrinous exudates abounding as well as both organizing and organized exudates. Perisplenitis was evident together with a covering of fibrinous exudates. These appearances were obvious on the liver, whose parenchyma was the seat of chronic venous congestion and incipient cirrhosis. The lung showed patchy edema and interstial pneumonia; a single localized area of eosinophilic granuloma was identified. Gynaecomastia was present. Apart from autolysis and congestion, the pancreas, thyroid and kidney revealed nothing noteworthy.

DISCUSSION

Although a 63 year old female sufferer was reported from Brazil (1), the commonest age ranges were described as “highest among persons 10 to 19 years of age” [5], “mainly children and adolescents” [6] or “predominantly children and young adults” [2]. Hence, our patient, aged 21 years, fits the above pictures.

Demise was usually due to heart failure [1,2,7,8]. It was prominent in our patient.

Marked ascites was noted in France [6]. So it was locally.

Intraventicular thrombus formation was generally a feature. This was linked with the valvulopathy seen in France [7]. Here, there was the specific “No thrombi were found.” If anything, there were “fibrinous exudates abounding as well as both organizing and organized exudates.”

CONCLUSION

Tropical endomyocardial fibrosis “remains a mysterious and challenging cardiovascular disease” [8]. Some answers have come from countries as far apart as Brazil (1), Casablanca (2), France (4), India (5), Mozambique (6,7) and the UK (8). Therefore, it is deemed to be important to add a Nigerian case; it is from the Ibo ethnic group [3]. In sum, forensic autopsy of a 21 year old man confirmed the existence of this odd disease in this African country. 

1.   Gutierrez PS, de Campos FPF (2017) Endomyocardial fibrosis. Autops Case Rep 7: 3-6.

2. Mocumbi AO, Yacoub MH, Yokohama H, Ferreira MB (2009) Right ventricular endomyocardial fibrosis. Cardiovasc Pathol 18: 64-65.

3.   Basden GT (1966) Niger Ibos. London: Cass.

4.  Macartney JC, Rollaston TP, Codling BW (1980) Use of a histopathology data pool for epidemiological analysis. J Clin Pathol 33: 351-355.

5.   Mocumbi AO, Ferreira MB, Sidi D, Yacoub MH (2008) A population study of endomyocardial fibrosis in a rural area of Mozambique. N Engl J Med 359: 43-49.

6.  Mocumbi AO (2012) Endomyocardial fibrosis: A form of endemic restrictive cardiomyopathy. Glob Cardiol Sci Pract 2012: 11.

7.  Huong DL, Wechsler B, Papo T, de Zuttere D, Bletry O, et al. (1997). Endomyocardial fibrosis in Behcet’s disease. Ann Rheum Dis 56: 205-208.

8.  Grimaldi A, Mocumbi AO, Freers J, Lachaud M, Mirabel M, et al. (2016) Tropical endomyocardial fibrosis. Natural history, challenges and perspectives. Circulation 133: 2503-2515.