Combined endothelin receptor antagonist and transcatheter interventional therapy of patent ductus arteriosus with severe pulmonary artery hypertension.
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.
Ussia
Gian Paolo
GP
Mulè
Massimiliano
M
Caruso
Elio
E
Aiello
Rosa
R
Tamburino
Corrado
C
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*
D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan