Adult Endocardial Fibroelastosis Associated with Dextrocardia and Situs Inversus

Abstract

The clinical and pathologic features of the first case of endocardial fibroelastosis in an adult, associated with dextrocardia and situs inversus, is reported. Difficulties in diagnosis are, evident. It is considered that the occurrence of this lesion in this patient is not, evidence of a congenital origin. Other intracardiac defects are rarely found associated with dextrocardia and situs inversus. The incidence of acquired heart disease in this group probably is no different from that in the general population. Endocardial fibroelastosis and its closely related entities in the adult may pose a difficult problem in differential diagnosis. The implications involved are realistic, since exploratory thoracotomy may be considered if constrictive pericarditis cannot be ruled out. This entity should be suspected in patients, particularly young adults, with progressive congestive failure where the etiology is obscure. Patients over 60 years of age with Stokes-Adams disease, in the absence of unequivocal evidence of arteriosclerotic heart disease or aortic stenosis, should also be suspected of having this entity. 33, 37 The frequent occurrence of thromboembolic episodes is more often associated with endocardial fibroelastosis than with other entities. Efforts to arrive at a definite diagnosis should be made, and exploration advised when constrictive pericarditis cannot be ruled out. Unfortunately, cases of endocardial fibrosis, myocardial fibrosis, and probably similar entities comprising the constrictive syndrome, on occasion will be found at operation. 59 This difficulty may arise if constrictive pericarditis is associated with cardiomegaly.