Worsening gradient of aortic stenosis with treatment of pulmonary arterial hypertension in scleroderma

Abstract

Systemic sclerosis (SSc) can cause interstitial lung and pulmonary vascular disease that can induce pulmonary arterial hypertension (PAH). It is well known that severe PAH may reduce left ventricluar preload and decrease diastolic filling with the potential of reducing forward flow. We present a case in which a patient with SSc and symptomatic PAH required direct pulmonary vasodilator therapy for treatment of elevated pulmonary pressures. On follow-up echocardiogram, while improvement in right ventricular function and reduction in estimated pulmonary pressures were noted; worsening of aortic valve gradients was also found. Cardiac hemodynamics of pulmonary vasodilator therapy is discussed and the literature is reviewed.