Idiopathic Restrictive Cardiomyopathy With Diffuse Perimyocytic Fibrosis

Idiopathic restrictive cardiomyopathy (RCM) is rare. Even today little is understood of its etiology or underlying mechanisms, and definitive diagnostic criteria are lacking. In this report, we describe a case of idiopathic RCM in a young Japanese woman who died while awaiting cardiac transplantation during the 5-year course of the disease. Rare pathologic findings of diffuse perimyocytic fibrosis were revealed at autopsy.