Abstract

Background and Objectives: Cardiac involvement is well recognized in cases of dermatomyositis (DM) and polymyositis (PM), with a variable frequency ranging from 9% to 72%. However, clinically significant heart involvement in DM/PM is relatively uncommon, and there are few reports of cardiac transplantation in DM. Our objectives were to describe a case of severe cardiac involvement in DM necessitating heart transplantation and to review the literature on cardiac involvement in DM and PM.

Methods: We describe a case of dermatomyositis in a patient with severe heart failure who underwent heart transplantation at our institution. Pathological examination of both the patient's explanted heart and skeletal muscle is reviewed. Additionally, a search of the MEDLINE database was conducted to identify reports of cardiac involvement in DM and PM.

Results: A 36-year-old man with DM presented with severe heart failure to our institution and underwent evaluation for heart transplantation. After a three-month hospitalization, he underwent successful cardiac transplantation. Pathological examination of his explanted heart revealed a pattern of inflammation and damage similar to that observed in DM in skeletal muscle. The patient is currently doing well, 20 months post-transplant, and is receiving maintenance therapy with tacrolimus, mycophenolate mofetil, rituximab, and low-dose prednisone. To our knowledge, this is the first case report of heart transplantation in dermatomyositis with analogous pathology observed in both cardiac and skeletal muscle.

Conclusions: Severe cardiac involvement necessitating transplantation is rare in dermatomyositis but can occur and appears to be related to a similar inflammatory process as observed in skeletal muscle.