Rapidly Progressive Myocarditis and Cardiogenic Shock: The Failing Heart in Systemic Sclerosis.

BACKGROUND

Cardiac involvement in systemic sclerosis (SSc) is under-recognized and may present as fulminant myocarditis with cardiogenic shock and poor outcomes.

CASE SUMMARY: A 49-year-old woman with diffuse SSc presented with progressive dyspnea and hypotension. Laboratory testing showed markedly elevated cardiac biomarkers, and echocardiography demonstrated severe biventricular systolic dysfunction. Electrocardiography revealed sinus tachycardia with right bundle branch block. Cardiac magnetic resonance showed diffuse biventricular myocardial inflammation, confirmed by endomyocardial biopsy demonstrating lymphocytic infiltration.

DISCUSSION: Despite inotropes, diuretic agents, and dual mechanical circulatory support with venoarterial extracorporeal membrane oxygenation and an Impella CP device, myocardial recovery did not occur. High-dose immunosuppression was delayed, and heart transplantation was deferred because of cavitary lung lesions. The patient developed progressive multiorgan failure and died in the sixth week of hospitalization.

TAKE-HOME MESSAGE: Fulminant SSc-myocarditis carries a grave prognosis and requires early recognition and co-ordinated multidisciplinary care.