Heart Failure with Preserved Ejection Fraction and Cardiomyopathy: an Under-recognized Complication of Systemic Sclerosis

Heart failure with preserved ejection fraction (HFpEF) and diastolic dysfunction are major sequelae of primary cardiac disease in patients with systemic sclerosis (SSc) and contribute to increased mortality in this population. Studies of animal models of SSc and human tissue samples indicate that the pathophysiology underlying these conditions involves microvascular dysfunction, cardiomyocyte apoptosis, and replacement fibrosis. Diagnostic recommendations for HFpEF in SSc include clinical examination and screening with laboratory studies and cardiac imaging to detect diastolic dysfunction early in the disease course. Management of HFpEF in this population does not significantly differ from general HFpEF guidelines. It is focused on symptomatic management of volume status and treatment of underlying SSc with immune-modulating therapies. Although cardiac disease is a major cause of mortality in patients with SSc, current treatments are lacking. There is a great need for novel therapeutics targeting the underlying pathophysiology in SSc to inhibit or reverse myocardial fibrosis, thus preventing cardiac remodeling and dysfunction in these patients.