Objective: To investigate the perioperative management of patients with hemophilia A (HA) who
undergo cardiac surgery.
Design
Retrospective analysis of the published literature.
Setting
University hospital.
Participants
HA patients undergoing cardiac surgery.
Interventions
The PubMed, Embase, Web of Science Core Collection, CNKI, VIP, and WANFANG databases
were searched with the terms “hemophilia A,” “cardiac surgery,” “cardiopulmonary bypass,”
“coronary artery bypass graft,” “valve disease,” and “congenital heart disease” to
identify relevant articles. Data regarding patient characteristics, perioperative
management protocols, and clinical outcomes were extracted and analyzed.
Measurements and Main Results
Fifty articles, with 72 patients aging from 12 days to 80 years, were included. The
analyzed population consisted of 36 (50%) mild HA, 10 (14%) moderate HA, and 25 (35%)
severe HA patients. Factor VIII inhibitors were detected in 7 cases. Surgical procedures
included 27 coronary artery bypass grafting procedures, 15 valve surgeries, 8 combined
coronary artery bypass grafting with valve surgery procedures (1 with left ventricular
assist device), 1 ventricular aneurysm resection with valve surgery, 11 corrections
of congenital heart disease, 7 adult aortic surgeries, 3 heart transplantations, and
1 repair of heart injury. Factor VIII concentrates were used as a substitution therapy
to ensure normalization of perioperative coagulation function. Although an uneventful
clinical outcome was achieved in the majority of cases, 20% of patients developed
complications with different severities.
Conclusions
Execution of a perioperative management strategy with a multidisciplinary approach,
a thorough factor replacement protocol, and careful monitoring of factor levels facilitate
an optimal outcome for HA patients undergoing cardiac surgery.
Key Words
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Article info
Publication history
Published online: July 25, 2020
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