title: Combined endothelin receptor antagonist and transcatheter interventional therapy of patent ductus arteriosus with severe pulmonary artery hypertension.
abstract: The natural history of congenital heart disease (CHD) with left to right shunt and high pulmonary blood flow, is characterized by development of severe Pulmonary Artery Hypertension (PAH); this condition usually contraindicates any type of surgical or interventional cardiac correction because of bad results. We here report the case of an adult patient with a patent ductus arteriosus and severe PAH, treated uneventfully with a staged combined therapy: Bosentan for 3 months and then percutaneous closure with amplatzer duct occluder (AGA Med. Co. Golden Valley, MN). The patient showed a dramatic improvement both of pulmonary hemodynamics and functional capacity at 8 months follow up confirming the efficacy of pulmonary vasodilator therapy in PAH associated to congenital heart disease.
authors.forename: Gian Paolo
authors.initials: GP
authors.lastname: Ussia
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Mulè
authors.forename: Elio
authors.initials: E
authors.lastname: Caruso
authors.forename: Rosa
authors.initials: R
authors.lastname: Aiello
authors.forename: Corrado
authors.initials: C
authors.lastname: Tamburino
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D006328:Cardiac Catheterization; D004374:Ductus Arteriosus, Patent / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D065128:Endothelin Receptor Antagonists; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D013449:Sulfonamides / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan