title: Long-term inhaled nitric oxide plus dipyridamole for pulmonary arterial hypertension.
abstract: Inhaled nitric oxide (iNO) has been shown to be a potent and selective vasodilator in pulmonary arterial hypertension (PAH). However, the clinical experience in prolonged treatment is limited. We assess the safety and effectiveness of long-term administration of iNO in severe PAH. Two female patients were admitted to our hospital because of severe dyspnea (World Health Organization functional class IV) and hypoxemia. They were diagnosed with PAH (primary and secondary to congenital heart disease) and treated with iNO for 2 years. The delivery system consisted of an NO tank of 800 ppm, a modified gas-pulsing device, and nasal cannulas. On iNO treatment the patients showed remarkable improvement of symptoms, oxygenation and 6-min walk distance. After 16 months the patients began to experience a progressive rebound of symptoms. A phosphodiesterase type 5 inhibitor (dipyridamole) was added to iNO. This intervention proved useful in improving clinical deterioration and hemodynamics. This is the first study reporting 2-year iNO therapy in 2 patients with primary and secondary pulmonary hypertension. The combination of dipyridamole with iNO augments the pulmonary vasodilatation and may be useful in managing PAH.
authors.affiliation: Pneumology Unit, Hospital Universitario de Gran Canaria Dr. Negrín, Las Palmas de Gran Canaria, Spain. gperpen@gobiernodecanarias.org
authors.forename: Gregorio
authors.initials: G
authors.lastname: Pérez-Peñate
authors.forename: Pedro
authors.initials: P
authors.lastname: Cabrera Navarro
authors.forename: Miguel
authors.initials: M
authors.lastname: Ponce González
authors.forename: Elías
authors.initials: E
authors.lastname: Górriz Gómez
authors.forename: Juan
authors.initials: J
authors.lastname: Pulido Duque
authors.forename: Antonio
authors.initials: A
authors.lastname: García Quintana
authors.forename: Gabriel
authors.initials: G
authors.lastname: Juliá Serdá
mesh_terms: D000280:Administration, Inhalation; D004176:Dipyridamole / Q000494:pharmacology / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D008875:Middle Aged; D009569:Nitric Oxide / Q000008:administration & dosage*; D010726:Phosphodiesterase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use*; D014664:Vasodilation / Q000187:drug effects; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D010726:Phosphodiesterase Inhibitors; D014665:Vasodilator Agents; D009569:Nitric Oxide; D004176:Dipyridamole