The patient presented in 1992 at age 28 years with syncope, palpitations, and worsening dyspnea on exertion. Her chest radiograph revealed cardiomegaly, and echocardiography ruled out an intracardiac shunt but revealed right heart enlargement. A workup was initiated that included ruling out an underlying connective tissue disorder with standard serology tests, a left and right heart catheterization to rule out congenital heart disease and left heart valvular disease, normal pulmonary function tests, a ventilation-perfusion (V̇/Q̇) scan to rule out thromboembolic disease, and a CT scan of the chest, which demonstrated normal lung parenchyma. The patient’s right heart catheterization revealed a pulmonary artery (PA) pressure of 82/44 mm Hg with a mean of 57 mm Hg, a right atrial pressure of 11 mm Hg, and a cardiac index of 2.65 L/min/m2. Primary pulmonary hypertension was diagnosed based on these results and the exclusion of other etiologies. A second cardiac catheterization was performed 5 months later and revealed slightly higher pressures with a PA pressure of 100/40 mm Hg (mean, 64 mm Hg) with a pulmonary capillary wedge pressure of 5 mm Hg. Her pulmonary vascular resistance was measured at 14.4 Wood units, and her cardiac index was 2.5 L/min/m2. She was treated with oral calcium channel blockers; however, her dyspnea worsened to the point of New York Heart Association class 3 symptomatology, and she was therefore referred for lung transplantation.