A Study of Thirty Cases

Subsequent to the definitive studies of Craig,’ Pryor and Wyatt,2 and others, endomyocardial fibroelastosis has been recognized as a distinct pathological entity even though certain details are not yet fully clarified. Several recent papers2-6 have brought knowledge of its clinical aspects to the point where it is almost as well defined clinically as pathologically. At first glance, it might seem that the least standardized facet of this syndrome is the nomenclature, for, among other terms, it is variously described as endocardial sclerosis or dysplasia, subendocardial sclerosis or fibrosis, endomyocardial fibrosis or fibroelastosis, depending, apparently, upon the author’s concept of the basic pathological process, which is not at all uniform or precisely defined. For the same reason, the last term will be used in this paper, in accord with the concept that the disease consists of a diffuse or focal thickening of the endocardium and the subendocardial myocardium, consisting predominantly of fibrous and elastic tissue. This is especially in accord with the concepts of Johnson.6 Study of those cases which have come to autopsy at Children’s Hospital (Denver), however, has led to the recognition of certain concepts which have apparently not been described in print, and others which have only just been recognized. This paper represents a study of the 30 cases discovered during the five year period ending April 30, 1952. The annual incidence curve resembles a logarithmic progression, indicating that this probably does not represent the total incidence in the earlier years of the period. Still earlier records, while yielding an occasional obvious case, are too incomplete to be of real value, especially since this disease was not then sought by the prosectors. The gross and microscopic pathology of all dubious cases were rechecked as closely as possible; the few in which this could not be satisfactorily accomplished are fully discussed in the appropriate section below. All cases in this study, with two exceptions, were diagnosed on the basis of the microscopic criteria of Craig,’ and Pryor and Wyatt.2 No microscopic examination Is available on these two cases. One of these (A48-2) is among the earliest of the series, and occurred at the time when interest In the entity was just developing. Grossly, it is a totally typical example of the atrio-ventricular valvular type of the disease as previously designated. The valves were described as follows: “The mitral and tricuspid valves are poorly developed. The mitral lacks chordae tendinae and papillary muscles. The edge of the tricuspid valve is nodular.”