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Frequency and severity of endocardial fibroelastosis in dilated hearts from children—endocardial thickness is inversely correlated to age

Published online by Cambridge University Press:  19 August 2008

Vera Demarchi Aiello  and
Maria de Lourdes Higuchi
Vera Demarchi Aiello*
Affiliation:
From the Serviço de Anatomia Patológica, Instituto do Corção do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo
Maria de Lourdes Higuchi
Affiliation:
From the Serviço de Anatomia Patológica, Instituto do Corção do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo
*
Dr. Vera D. Aiello, Instituto do Coração, HC FMUSP, Serviço de Anatomia Patológica, Avenida Dr. Enéas C. Aguiar 44, 05403-000 São Paulo, SP, Brazil.
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Summary

Diffuse endocardial thickening in dilated hearts has been considered as a distinct pathological entity, usually being termed endocardial fibroelastosis. It is common in infants and children and rare in adults. Current opinion holds that the endocardial thickening is a non-specific reaction to stress in the ventricular wall. Our personal observations point to the most severe forms occurring in young infants. To determine the frequency and severity of endocardial fibroelastosis in children with dilated cardiomyopathy, we studied 21 necropsy hearts, both grossly and microscopically. Ages of the patients ranged from two to 175 months (mean 46.5, median 23 months). Using a graduated eyepiece, we measured the endocardial thickness in samples from three sites in the left ventricle and one from the right ventricle. The values were compared to those obtained from similar measurements of the endocardium in hearts from four children presenting with tetralogy of Fallot. All the dilated hearts presented some degree of endocardial thickening. The gross detection of fibroelastosis depended on the degree of thickening. There was a negative but linear correlation between age and the mean endocardial thickness (r=0.70; p>0.001). Our findings support the concept that endocardial cells have greater replicative and secretory potentials in young patients, and that endocardial fibroelastosis is a secondary phenomenon in dilated hearts.

Keywords

Endocardial fibroelastosisdilated cardiomyopathyprimary and secondary cardiomyopathy
Type
Original Articles
Information
Cardiology in the Young , Volume 4 , Issue 2 , April 1994 , pp. 117 - 121
Copyright
Copyright © Cambridge University Press 1994

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References

1.Maron, BJ. Cardiomyopathies. In: Moss, AJ, Adams, FH (eds). Heart Diseases in Infants, Children and Adolescents. Third edition. Williams and Wilkins Co., Baltimore, 1983, pp 940–964.Google Scholar
2.Brandenberg, RO, Chazov, E, Cherian, G, Falase, AO, Grosgegeat, Y, Kawai, C, Loogen, F, Martin Judez, V, Orinius, E, Goodwin, JF, Olsen, EGJ, Oakley, CM, Pisa, Z. Report of the WHO/Isfc task force on definition and classification of cardiomyopathies. Circulation 1981; 64: 437A–438A.Google Scholar
3.Lurie, PR. Endocardial fibroelastosis is not a disease. Am J Cardiol 1988; 62: 468–470.CrossRefGoogle Scholar
4.Hutchins, GM, Vie, SA. The progression of interstitial myocarditis to idiopathic endocardial fibroelastosis. Am J Pathol 1972; 66: 483–491endpage.Google ScholarPubMed
5.Westwood, M, Harris, R, Burn, JL, Barson, AJ. Heredity in primary endocardial fibroelastosis. Br Heart J 1975; 37: 1077–1084.CrossRefGoogle ScholarPubMed
6.Hanukoglu, A, Fried, D, Somekh, E. Inheritance of familial endocardial fibroelastosis. Clin Pediatr 1986; 25: 272–275.CrossRefGoogle Scholar
7.Stephan, MJ, Stevens, EL Jr, Wenstrup, RJ, Greenberg, CR, Gritter, HL, Hodges, GF, Guller, B. Mucopolysaccharidosis I presenting with endocardial fibroelastosis of infancy. Am J Dis Child 1989; 143: 782–784.Google ScholarPubMed
8.Stamato, NJ, O'Connell, JB, Subramanian, MB, Scanlon, PJ. Diagnosis of endocardial fibroelastosis by endomyocardial biopsy in an adult with dilated cardiomyopathy. Am Heart J 1985; 109: 919–920.CrossRefGoogle Scholar
9.Ino, T, Benson, LN, Freedom, RM, Rowe, RD. Natural history and prognostic risk factors in endocardial fibroelastosis. Am J Cardiol 1988; 62: 431–434.CrossRefGoogle ScholarPubMed
10.Kline, IK, Miller, AJ, Pick, R, Katz, LN. The relationship between human endocardial fibroelastosis and obstruction of the cardiac lymphatics. Circulation 1964; 30: 728–735.CrossRefGoogle ScholarPubMed
11.Factor, SM. Endocardial fibroelastosis: Myocardial and vascular alterations associated with viral like nuclear particles. Am Heart J 1978; 96: 791–801.CrossRefGoogle ScholarPubMed
12.Okada, R. Clinicopathological study on the thickening of parietal endocardium in the adult heart. Jap Heart J 1961; 2: 220–255.CrossRefGoogle Scholar
13.Griffin, ML, Hernandez, A, Martin, TC, Goldring, D, Bolman, RM, Spray, TL, Strauss, AW. Dilated cardiomyopathy in infants and children. J Am Coll Cardiol 1988; 11: 139–144.CrossRefGoogle ScholarPubMed
14.Angelov, AM, Kulova, A, Gurdevsky, M. Endocardial fibroelastosis. Clinico study of 38 cases. Path Res Pract 1984; 178: 384–388.CrossRefGoogle ScholarPubMed
15.Carceller, AM, Maroto, E, Fouron, JC. Dilated and contracted forms of primary endocardial fibroelastosis: a single fetal disease with two stages of development. Brit Heart J 1990; 63: 311–313.CrossRefGoogle ScholarPubMed
16.Sharland, GK, Chita, SK, Fagg, NL, Anderson, RH, Tynan, M, Cook, AC, Allan, LD. Left ventricular dysfunction in the fetus: relation to aortic valve anomalies and endocardial fibroelastosis. Br Heart J 1991; 66: 419–424.CrossRefGoogle ScholarPubMed