title: Pulmonary thromboembolism superimposed on a congenital ventricular septal defect in a 50-year-old man inhaled nitric oxide and sildenafil to the rescue.
abstract: We report the combined use of inhaled nitric oxide (iNO) and sildenafil to selectively lower pulmonary vascular resistance (PVR) and reverse right-to-left shunt flow across a congenital ventricular septal defect (VSD) in a 50-year-old man. The patient developed right-to-left shunt flow with profound hypoxia because of an acute pulmonary embolism superimposed on underlying pulmonary hypertension. The dramatic improvement in oxygenation and PVR with sildenafil plus iNO has been sustained with sildenafil monotherapy. To our knowledge, use of this combination therapy in patients with decompensated pulmonary arterial hypertension (PAH) and a predisposition to right-to-left shunting has not been previously reported.
authors.affiliation: Division of Cardiology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114, USA.
authors.forename: Gregory D
authors.initials: GD
authors.lastname: Lewis
authors.forename: Kenneth D
authors.initials: KD
authors.lastname: Bloch
authors.forename: Marc J
authors.initials: MJ
authors.lastname: Semigran
mesh_terms: D000280:Administration, Inhalation; D000284:Administration, Oral; D001993:Bronchodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use; D004452:Echocardiography; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009569:Nitric Oxide / Q000008:administration & dosage / Q000627:therapeutic use; D010879:Piperazines / Q000008:administration & dosage / Q000627:therapeutic use; D011655:Pulmonary Embolism / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D011687:Purines; D000068677:Sildenafil Citrate; D013450:Sulfones; D014655:Vascular Resistance / Q000187:drug effects; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D001993:Bronchodilator Agents; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D009569:Nitric Oxide; D000068677:Sildenafil Citrate