Use of Oral Sildenafil in Patients With Irreversible Pulmonary Hypertension Not Eligible for Heart Transplantation

Abstract

Heart transplantation is contraindicated in patients with acute irreversible pulmonary hypertension (PH), but new drugs are opening up therapeutic possibilities. Sildenafil citrate is a nonselective pulmonary vasodilator that is being used in our hospital to treat several patients with PH and which has allowed the inclusion of 1 patient on the waiting list for heart transplantation. A 20-year-old man with Becker muscular dystrophy was diagnosed at the age of 19 years with dilated cardiomyopathy with severe pulmonary artery systolic pressure (PH = 60 mm Hg). A pretransplantation study, including a right hemodynamic analysis with an acute vasodilator test using intravenous epoprostenol, revealed the irreversible character of the PH. Inasmuch as the administration of dobutamine did not achieve an adequate reduction of PH, oral sildenafil was started (25 mg every 12 hours) as salvage therapy. An echocardiogram obtained 2 months after starting sildenafil therapy showed normal right cavities, previously dilated, as well as minimal protosystolic tricuspid regurgitation without PH. A new right hemodynamic study performed after 4 months showed a reduction in pulmonary vascular resistance, from 8 U to 3.5 U Woods. As a result, the patient has now been included on the waiting list for heart transplantation. The promising example of this patient confirms the necessity to carry out controlled trials to establish definitively the indications for the use of sildenafil in patients with irreversible PH.