Depressed LV Contractility as a Sentinel of Fulminant Systemic Scleroderma

Depressed left ventricular (LV) contractility is rare in cardiac scleroderma. We present a case of rapidly progressive systemic scleroderma (SSc) with decreased LV function.

A previously healthy 21-year-old man presented with palpitations, weight loss, and diffuse swelling. Evaluation revealed reduced LV function, diffuse gadolinium enhancement on cardiac magnetic resonance imaging, and endomyocardial biopsy showed mast cell infiltration and early fibrosis. Despite initiation of guideline-directed medical therapy, cardiac function deteriorated over 1 month. Further workup established SSc. Cyclophosphamide was initiated; however, the patient succumbed to a saddle pulmonary embolism 2 months after diagnosis.

LV systolic dysfunction in SSc signals advanced myocardial involvement and demands urgent recognition, as deterioration may occur within a narrow therapeutic window. Early detection and intervention are critical to improving outcomes in this vulnerable subset of patients.

Patients with depressed LV contractility in cardiac scleroderma should receive prompt cardiac testing and treatment.