title: Bosentan induces clinical, exercise and hemodynamic improvement in a pre-transplant patient with plastic bronchitis after Fontan operation.
abstract: We present a 14-year-old pre-transplant boy with elevated pulmonary pressure and plastic bronchitis after Fontan operation despite stent fenestration, who improved with long-term oral administration of the endothelin antagonist, bosentan. Bosentan improved the patient's symptoms and aortic saturation, World Health Organization class, maximal and sub-maximal exercise capacity, Borg dyspnea index, invasively measured mean pulmonary pressure, pulmonary blood flow and pulmonary vascular resistance. This is the first literature report examining the effect of endothelin antagonism in failing Fontan circulation and showing clinical, exercise and hemodynamic improvement with bosentan in a pre-transplant Fontan patient with high pulmonary pressure and plastic bronchitis.
authors.affiliation: Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, Athens, Greece. riapos@hol.gr
authors.forename: Sotiria C
authors.initials: SC
authors.lastname: Apostolopoulou
authors.forename: John
authors.initials: J
authors.lastname: Papagiannis
authors.forename: Spyridon
authors.initials: S
authors.lastname: Rammos
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000601:surgery*; D000293:Adolescent; D000077300:Bosentan; D001991:Bronchitis / Q000175:diagnosis / Q000188:drug therapy*; D017079:Exercise Tolerance / Q000187:drug effects; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects* / Q000379:methods; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D016027:Heart Transplantation; D006439:Hemodynamics / Q000187:drug effects / Q000502:physiology; D006801:Humans; D008297:Male; D011300:Preoperative Care; D016459:Prosthesis-Related Infections / Q000175:diagnosis / Q000188:drug therapy*; D018570:Risk Assessment; D013449:Sulfonamides / Q000008:administration & dosage*; D016896:Treatment Outcome; D014850:Waiting Lists
chemical_list: D013449:Sulfonamides; D000077300:Bosentan