Adult Fibroelastosis with Congenital Tricuspid Stenosis

Abstract

A 39-year-old male was observed for 3 years with progressive cyanotic cardiac disease, beginning with repeated peripheral embolization, progressing to congestive heart failure and fever and prominent ascites. Ecg changes suggested pericarditis or anoxia. Cardiac catheterization revealed normal pulmonary arterial pressure and high right atrial pressure with "M" curve and plateau. Operation for incision of infundibular stenosis effected no improvement and the course terminated in shock and congestive heart failure. Clinically the condition was diagnosed as congenital tricuspid stenosis. Pathologic diagnosis was fibroelastosis with congenital tricuspid stenosis due to congenital disorganization of endothelium, hypoplasia of right ventricular inflow tract, myocardial fibrosis and mural thrombosis, rheumatic mitral and aortic valvulitis, cardiac cirrhosis and terminal SBE.