Management of Patients With Hemophilia Undergoing Cardiac Surgery

Excellent hemostatic control during cardiac surgery in a patient with hemophilia B treated with albutrepenonacog alfa (rIX‐FP): A case report

“…The use of aPTT during cardiopulmonary bypass is not feasible, as heparin concentrations of >1 U/mL will disrupt aPTT measurements, while FVIII or FIX assays cannot be performed quickly enough during cardiac surgery. 26 In summary and to the best of our knowledge, this is the first report of cardiac surgery with ECC in a patient with hemophilia B receiving treatment with rIX-FP. Based on the success of this patient's surgery and the suggestions of other authors regarding cardiac surgery in patients with hemophilia, we consider that there are a series of considerations that are essential for the management of this type of patient in these situations: (1) surgery should be scheduled under the supervision of a multidisciplinary team with experience in this field, (2) treatment should be individualized following current WHO recommendations, (3) treatment should be closely monitored to ensure good hemostatic protection during and after surgery, (4) viscoelastic testing during the perioperative process is very useful to assess the patient's overall hemostatic status, and (5) treatment with EHL FIX, such as rIX-FP, in patients with HB is safe and effective in this type of surgery.…”

“…Indeed, reports of cardiac surgery in patients with hemophilia, especially hemophilia B, are very scarce, and are limited to case reports, small series, or expert opinions. Most recent reviews have focused on perioperative management but fail to address optimal strategies for the management of these patients during surgery 26–28 …”

“…Viscoelastic techniques are thus positioned as a plausible alternative to conventional techniques such as activated partial thromboplastin time (aPTT) and specific assays for FVIII and FIX. The use of aPTT during cardiopulmonary bypass is not feasible, as heparin concentrations of >1 U/mL will disrupt aPTT measurements, while FVIII or FIX assays cannot be performed quickly enough during cardiac surgery 26 …”

“…Most recent reviews have focused on perioperative management but fail to address optimal strategies for the management of these patients during surgery. [26][27][28] Cardiac surgery with and without ECC has been shown to be effective and safe. However, in expert hands, surgery without ECC has been shown to reduce morbidity and, in nonrandomized adjusted studies, mortality in high-risk patients.…”

“…Most experts agree that cardiac surgery in patients with hemophilia requires a multidisciplinary and highly individualized approach for each patient, with the participation of hematologists, anesthesiologists, surgeons, intensivists, and laboratory specialists. 26,28 It should be noted that this patient's surgery was scheduled for a Monday, because, as recommended by some authors, performing surgery at the beginning of the week helps to ensure the availability of all personnel and thus more satisfactory patient outcomes. [30][31][32] With regard to therapeutic interventions during the surgical process, we found that optimization of factor levels before, during and after surgery was essential to obtain results similar to those obtained in non-hemophiliac patients.…”