Abstract
Endomyocardial Fibrosis (EMF) is a cardiomyopathy with high prevalence in Sub-Saharan Africa with unclear etiology, pathogenesis and natural history. Most pathological abnormalities can potentially be diagnosed by echocardiography allowing surgery in some cases. With increasing availability of echocardiography in endemic areas for EMF we designed a study aiming at assessing its accuracy in defining EMF structural abnormalities pre-operatively, and describe pathological findings through detailed intraoperative examination and evaluation of histopathological changes in tissue obtained from excisional biopsies. Transthoracic echocardiography was performed pre-operatively to 29 patients with chronic severe EMF. All patients were submitted to excisional endomyocardial biopsies during surgery and careful standardized intra-operative evaluation was also done. Surgical and histopathological findings were compared to those obtained by echocardiography. Of the 29 patients studied, 16 had moderate lesions while 13 had severe disease. Their mean age was 12 (±4.6) years and 17 were males. All but one patient were in NYHA functional class III or IV at time of surgery. All patients had severe atrioventricular valve regurgitation with valves considered suitable for repair. We evaluated tissue from 25 left ventricles and 12 right ventricles. Endocardial thickening was the most prominent abnormality due to deposition of hyaline and cellular collagen underneath a layer of apparently normal endocardial endothelial cells. The mean endocardial thickness was 2,541 ± 1,707 μm. There was high agreement between echocardiographic and intraoperative findings, with the findings coinciding completely in 24 patients. Severe EMF assessed by echocardiography was associated with intense endocardial fibrosis on histology. In contrast, the presence of inflammation in peripheral blood was not associated with tissue inflammation. Structural abnormalities of chronic severe EMF are accurately diagnosed by transthoracic echocardiography, allowing this non-invasive technique to be used as the gold standard for diagnosis and surgical management of chronic EMF in endemic areas.
Similar content being viewed by others
References
Mocumbi AO, Ferreira MB, Sidi D, Yacoub MH (2008) A population study of endomyocardial fibrosis in a rural area of Mozambique. N Eng J Med 369:43–49
Iglezias S, Benvenuti L, Calabrese F et al (2008) Endomyocardial fibrosis: pathological and molecular findings of surgically resected ventricular endomyocardium. Virchows Arch 453:233–241
de Oliveira SA, Pereira Barreto AC, Mady C, Dallan LA, da Luz PL, Jatene AD, Pileggi F (1990) Surgical treatment of endomyocardial fibrosis: a new approach. J Am Coll Cardiol 16(5):1246–1251
Connor DH, Somers K, Hutt NSR, Manion WC, D’Arbela PGD (1967) Endomyocardial fibrosis in Uganda (Davies’ disease) Part I. Am Heart J 74(5):687–709
Connor DH, Somers K, Hutt NSR, Manion WC, D’Arbela P (1968) Endomyocardial fibrosis in Uganda (Davies’ disease) Part II. Am Heart J 74(5):107–124
Shaper AG, Hutt MSR, Coles RM (1968) Necropsy study of endomyocardial fibrosis and rheumatic heart disease in Uganda 1950–1965. Brit Heart J 30:391–401
Chopra P, Narula J, Talwar KK, Kumar V, Bhatia ML (1990) Histomorphologic characteristics of endomyocardial fibrosis: an endomyocardial biopsy study. Hum Pathol 21(6):613–616
Andrade ZA, Teixeira ARL (1973) Changes in the vasculature in endomyocardial fibrosis and their possible significance. Am Heart J 86:152–158
Lira VM (1996) Endomyocardial fibrosis pathology. Arq Bras Cardiol 67(4):273–277
Santos CL, Moraes CR, Santos FL et al (2001) Endomyocardial fibrosis in children. Cardiol Young 11(2):205–209
Saraiva LR, Carneiro RW, Arruda MB, Brindeiro D, Lira V (1999) Mitral valve disease with rheumatic appearance in the presence of left ventricular endomyocardial fibrosis. Arq Bras Cardiol 72(3):330–332
Cheung SC, Chan CWS. (2008) Insights of prognostication of Davies disease: What could we learn from serial magnetic resonance imaging studies? Int J Cardiol (epub PMID: 19144428)
Decker B, Sender S, Gros G (1996) Membrane-associated carbonic anhydrase IV in skeletal muscle-subcellular localization. Histochem Cell Biol 106:405–411
Frustaci A, Abdual AK, Possati G, Manzoli U (1989) Persisting hypereosinophilia and myocardial activity in the fibrotic stage of endomyocardial disease. Chest 96:674–675
Ball JD, Williams AW, Davies JNP (1954) Endomyocardial fibrosis. Lancet 1:1049–1054
Edington GM, Gilles HM (1976) Pathology in the tropics. Edward Arnold Publishers, London, pp 357–365
Jaiyesimi F (1982) Controversies and advances in endomyocardial fibrosis: a review. Afr J Med Med Sci 11:37–46
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Mocumbi, A.O., Carrilho, C., Sarathchandra, P. et al. Echocardiography accurately assesses the pathological abnormalities of chronic endomyocardial fibrosis. Int J Cardiovasc Imaging 27, 955–964 (2011). https://doi.org/10.1007/s10554-010-9753-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10554-010-9753-6