Aortic valve replacement in a patient with erythropoietic protoporphyria.
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.
Yotsumoto
Goichi
G
Second Department of Surgery, Kagoshima University, Faculty of Medicine, Kagoshima, Japan. yotumoto@m2.kufm.kagoshima-u.ac.jp
Masuda
Hiroshi
H
Iguro
Yoshifumi
Y
Kinjo
Tamahiro
T
Matsumoto
Hitoshi
H
Sakata
Ryuzo
R
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
D011524:Protoporphyrins; D006418:Heme