A 55-year-old woman, nonsmoker, with group 1 pulmonary arterial hypertension (PAH) secondary to systemic lupus erythematous, New York Heart Association class II, was hospitalized for 1 day of worsening exertional dyspnea. She had associated intermittent substernal chest pain with radiation to the left arm that was aggravated by coughing and movement. The patient also had one syncopal episode while at rest. Current treatment of PAH included sildenafil, warfarin, and participation in the double-blind placebo-controlled Prostacyclin (PGI2) Receptor Agonist in Pulmonary Arterial Hypertension (GRIPHON) trial investigating selexipag, an oral prostacyclin receptor analog. Prior therapies included inhaled treprostinil, inhaled iloprost, and subcutaneous treprostinil, all discontinued because of intolerance of side effects.