title: Aortic valve replacement in a patient with erythropoietic protoporphyria.
abstract: Erythropoietic protoporphyria (EPP) is a disorder of heme synthesis that causes excessive accumulation of protoporphyrin. The predominant clinical feature is photosensitivity triggered by light at wavelengths near 400 nm. We describe a 52-year-old man with EPP who underwent aortic valve replacement due to severe regurgitation. To prevent burn injuries, astral lamps in the operating room were covered with yellow film filters. Preoperative autologous blood donation was not undertaken. Blood priming of the extracorporeal circuit was performed to maintain adequate hemoglobin concentrations, which resulted in reduction of heme synthesis. The patient was discharged in good health without any signs or symptoms of EPP.
authors.affiliation: Second Department of Surgery, Kagoshima University, Faculty of Medicine, Kagoshima, Japan. yotumoto@m2.kufm.kagoshima-u.ac.jp
authors.forename: Goichi
authors.initials: G
authors.lastname: Yotsumoto
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Masuda
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Iguro
authors.forename: Tamahiro
authors.initials: T
authors.lastname: Kinjo
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Matsumoto
authors.forename: Ryuzo
authors.initials: R
authors.lastname: Sakata
mesh_terms: D001021:Aortic Valve / Q000601:surgery*; D001022:Aortic Valve Insufficiency / Q000097:blood / Q000601:surgery*; D002315:Cardiopulmonary Bypass; D017707:Erythrocyte Transfusion; D005374:Filtration / Q000295:instrumentation; D006418:Heme / Q000096:biosynthesis; D006452:Hemoglobinometry; D006801:Humans; D008029:Lighting / Q000295:instrumentation; D008297:Male; D008875:Middle Aged; D009873:Operating Rooms; D017121:Porphyria, Hepatoerythropoietic / Q000097:blood / Q000601:surgery*; D011524:Protoporphyrins / Q000097:blood; D012307:Risk Factors
chemical_list: D011524:Protoporphyrins; D006418:Heme