A 64-year-old woman presented with dyspnea for a year. She was diagnosed with systemic sclerosis (SSc) in 2014 and no pulmonary and cardiovascular complications were observed until 2020. The dyspnea onset in early 2021 led her to a cardiologist and transthoracic echocardiography showed biventricular dilatation and a left ventricular ejection fraction of 20%. Cardiac magnetic resonance imaging (CMRI) showed extensive late gadolinium enhancement (LGE) subendocardium of left ventricular walls, which suggested multi-vessel coronary artery occlusion (Figure 1A-D). However, there was no significant epicardial coronary artery obstruction on coronary angiography (Figure 1E and F). One year later, she was admitted for aggravating dyspnea despite guideline-directed medical therapy and became dobutamine dependent state. The right heart catheterization showed biventricular systolic dysfunction, but no pulmonary hypertension was noted (pulmonary artery pressure, systolic/diastolic/mean: 27/16/19 mmHg, pulmonary vascular resistance: 1.8 wood unit). She received a heart transplant on day 53 after hospitalization. Pathologic examination of the explanted heart revealed severe endocardial fibrosis and multifocal patch fibrosis of the myocardium in both ventricles (Figure 2). Furthermore, severe microvascular disease was noted. Most intracardiac arteries including atrioventricular nodal artery and arterioles showed fibromuscular dysplastic changes with luminal narrowing (Figure 3) consistent with ischemic pattern LGE in CMRI. Similar to the previous report,1) the principal pathogenic process in this SSc case was the extensive microvascular occlusion, leading to distinguished ischemic cardiomyopathy from atherosclerotic ischemia.