title: Anesthetic management for combined mitral valve replacement and aortic valve repair in a patient with osteogenesis imperfecta.
abstract: Osteogenesis imperfecta is a rare disorder of connective tissues and presents multiple challenges, including difficult airway, hyperthermia, coagulopathy and respiratory dysfunction, for anesthesiologists, especially during cardiac surgery. We present anesthetic management of a patient with osteogenesis impertecta during double valve surgery. Dexmedetomidine infusion minimized the risks of malignant hyperthermia. Glidescope and in-line stabilization facilitated endotracheal intubation and protected his oral structures and cervical spine. Transesophageal echocardiography (TEE) diagnosed a flail A3 segment and redundant left coronary cusp causing mitral and aortic regurgitation. The mitral valve was replaced and the aortic valve repaired. Coagulopathy was corrected according to comprehensive coagulation analysis. Glidescope, dexmedetomidine, coagulation analysis and TEE could facilitate anesthetic management in these patients.
authors.affiliation: Department of Anesthesia, Jewish Hospital and St. Mary's Healthcare, Louisville, KY, USA. jiapenghuang@yahoo.com
authors.forename: Jiapeng
authors.initials: J
authors.lastname: Huang
authors.forename: Michelle
authors.initials: M
authors.lastname: Dinh
authors.forename: Nicholas
authors.initials: N
authors.lastname: Kuchle
authors.forename: Jing
authors.initials: J
authors.lastname: Zhou
mesh_terms: D000732:Androstanols; D000758:Anesthesia; D018686:Anesthetics, Intravenous; D001021:Aortic Valve / Q000601:surgery*; D001778:Blood Coagulation Disorders / Q000188:drug therapy; D020680:Bronchoscopes; D002208:Caproates / Q000627:therapeutic use; D002315:Cardiopulmonary Bypass; D056805:Consciousness Monitors; D020927:Dexmedetomidine; D017548:Echocardiography, Transesophageal; D005283:Fentanyl; D006333:Heart Failure / Q000209:etiology; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006801:Humans; D006993:Hypnotics and Sedatives; D007442:Intubation, Intratracheal / Q000379:methods; D008297:Male; D008305:Malignant Hyperthermia / Q000517:prevention & control; D008943:Mitral Valve / Q000601:surgery*; D008944:Mitral Valve Insufficiency / Q000601:surgery; D003473:Neuromuscular Nondepolarizing Agents; D010013:Osteogenesis Imperfecta / Q000150:complications*; D010976:Platelet Count; D000077123:Rocuronium; D055815:Young Adult
chemical_list: D000732:Androstanols; D018686:Anesthetics, Intravenous; D002208:Caproates; D006993:Hypnotics and Sedatives; D003473:Neuromuscular Nondepolarizing Agents; D020927:Dexmedetomidine; D005283:Fentanyl; D000077123:Rocuronium