[Aortic root replacement in Marfan syndrome with hemophilia A].
A 34-year-old man with Marfan syndrome was admitted to our hospital for surgical treatment of aortic regurgitation due to annuloaortic ectasia. He had no history of bleeding complications. Preoperative investigation revealed a slight prolongation of an activated partial thromboplastin time, which went unnoticed. He underwent aortic root replacement with a composite valve graft. During the operation, he had excessive bleeding due to coagulopathy after the termination of cardiopulmonary bypass, and needed a large amount of blood transfusion to obtain hemostasis. Before his discharge from our hospital, he was diagnosed as mild hemophilia A because of the decline in his factor VII level. To our knowledge, there has been no published case of cardiac operations in Marfan syndrome with hemophilia A.
Kumano
H
H
Department of Cardiovascular Surgery, Osaka City University Medical School, Osaka, Japan.
Shibata
T
T
Sasaki
Y
Y
Hirai
H
H
Hosono
M
M
Fukui
T
T
Fujii
H
H
Arimoto
H
H
Motoki
M
M
Bito
Y
Y
Takahashi
Y
Y
Suehiro
S
S
D000328:Adult; D001021:Aortic Valve / Q000601:surgery*; D001022:Aortic Valve Insufficiency / Q000601:surgery*; D019918:Heart Valve Prosthesis Implantation; D006467:Hemophilia A / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000601:surgery*