Isolated right ventricular failure in scleroderma heart disease

Scleroderma is reported to have numerous cardiac manifestations. Right ventricular failure (RVF) is a well-recognized cardiac complication of scleroderma and most often is related to underlying pulmonary hypertension (PH). Causes of PH include both interstitial lung disease and pulmonary artery vasculopathy. Direct involvement of the ventricle by myocardial fibrosis of coronary vasospasm could also cause a diffuse bilateral cardiomyopathy. We describe a case of predominant RVF in the absence of significant PH in a patient with longstanding scleroderma.