Hemophilia: A Review of Perioperative Management for Cardiac Surgery

Management of Patients With Hemophilia Undergoing Cardiac Surgery

“…affecting only about 34,000 people worldwide. 2,3 Hemophilia severity is classified based on factor activity level as mild (5-40%), moderate (1-5%), or severe (<1%). 4 Patients with mild hemophilia are often asymptomatic and become coagulopathic only after massive trauma or major surgery.…”

“…However, they are still at relevant risk of atherosclerotic vascular lesions and degenerative valvular disease with advanced age, which generally can be expected with recent therapeutic improvements. 2,5 Accordingly, hemophilia patients increasingly present for cardiac surgery.…”

“…Recent reviews have increased the insights in perioperative management. 1,2,6 However, the evidence for optimal strategy is limited to small case series and experts' opinions. The case report by Rajasekhar and colleagues in this issue of the JOURNAL OF CARDIOTHORACIC AND VASCULAR ANESTHESIA is another piece of the puzzle in the perioperative management for hemophilia patients undergoing cardiac surgery.…”

“…Multiple plasma-derived or recombinant FVIII and FIX replacement products are available. 1,2,6,7 The half-lives of different FVIII and FIX concentrates are between 8-12 hours and 18-34 hours, respectively. Recently, products with extended half-lives by fusion, conjugation or other protein modification have been released.…”

“…8 Several replacement protocols for hemophilia patients undergoing cardiac surgery have been published, usually with low numbers of included patients. 1,2,6 Generally, replacement protocols target a FVIII activity of 80 to 100% before, during and early after surgery. A typical regimen consists of a bolus of 45 to 50 U/kg before induction of anesthesia, followed by a bolus of about 2000 U after separation from CPB resulting in a FVIII concentration of >80% of normal activity during and after surgery.…”