Replacement myocardial fibrosis at the site of late gadolinium enhancement on magnetic resonance imaging in a patient with diffuse cutaneous systemic sclerosis: An autopsy report

Abstract

Late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) is a well-known finding indicative of cardiac involvement in systemic sclerosis (SSc heart). However, few studies have reported the precise histopathology at the site of LGE. We present an autopsy report of a 51-year-old man diagnosed with diffuse cutaneous SSc according to a systematic diagnostic workup, including skin biopsy. CMR indicated left ventricular (LV) dilatation and broadly distributed subendocardial LGE in the LV walls. The patient was treated with methylprednisolone pulse therapy because of multiple episodes of ventricular tachycardia, whereas he subsequently died of left heart failure. An autopsy study revealed broad subendocardial replacement fibrosis, concomitant with the distribution of LGE on CMR, without inflammatory or edematous changes. Notably, myocardial fibrosis was evident around the intramural coronary arteries, although the arteries themselves were intact. These findings demonstrated that broad subendocardial LGE on CMR reflected replacement myocardial fibrosis in a patient with diffuse cutaneous SSc. These clinicopathological observations suggested that spasms in the intramyocardial arteries or the cardiac Raynaud’s phenomenon may have provoked broad subendocardial fibrosis of the LV walls.<Learning objective: The present autopsy report pathologically validated the presence of broad myocardial fibrosis in the area of subendocardial late gadolinium enhancement (LGE) on cardiac magnetic resonance in a patient with systemic sclerosis (SSc). Lack of inflammatory changes along with intact coronary arteries suggested the involvement of intramural coronary spasm in the development of cardiac involvement in SSc. Such an LGE pattern may suggest end-stage cardiac involvement and portend a poor prognosis.>