title: [Anesthetic management of a patient with Osler-Weber-Rendu disease undergoing mitral valvuloplasty].
abstract: Osler-Weber-Rendu disease (OWRd) is an autosomal dominant disease with an incidence of 1-2 per 100,000 of population. The triad of OWRd is fibrovascular dysplasia characterized by telangiectases, arteriovenous malformations and recurrent bleeding. We experienced anesthetic management of a patient with OWRd undergoing mitral valvuloplasty. The patient was a 79-year-old woman admitted to our hospital with diagnosis of heart failure, mitral regurgitation, and chronic atrial fibrillation. Massive nasal bleeding before admission caused severe anemia (Hb 4.8 g dl(-1)), and she received transfusion. Preoperaitve examination revealed moderate mitral regurgitation with prolapsed anterior leaflet, pulmonary hypertension, high cardiac output (6.85 l min(-1)), and multiple hepatic angiomas. After induction of anesthesia, a pulmonary catheter was inserted. High cardiac output and high saturation of mixed-venous oxygen were found until the end of surgery. Blood gas analyses of samples from several sites suggested left to right shunt below the inferior vena cava. Mitral valvuloplasty and tricuspid annuloplasty were completed under mild hypothermic cardiopulmonary bypass. Despite our attention to abnormal bleeding, perioperative course was uneventful. For anesthetic management of OWRd, we must recognize the abnormal bleeding and the systemic vascular malformations preoperatively, and prepare for the possible complications.
authors.affiliation: Department of Anesthesiology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan.
authors.forename: Woonkwan
authors.initials: W
authors.lastname: Hyun
authors.forename: Masahiro
authors.initials: M
authors.lastname: Gamo
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Hirose
mesh_terms: D000368:Aged; D000758:Anesthesia; D001281:Atrial Fibrillation / Q000150:complications; D005260:Female; D006333:Heart Failure / Q000150:complications; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications; D008943:Mitral Valve / Q000601:surgery*; D008944:Mitral Valve Insufficiency / Q000150:complications / Q000601:surgery*; D019990:Perioperative Care; D011667:Pulmonary Veins / Q000601:surgery; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*; D014261:Tricuspid Valve / Q000601:surgery