PB1841 ENDOVASCULAR ABDOMINAL AORTIC ANEURYSM REPAIR IN A MILD HAEMOPHILIA A PATIENT: A CASE REPORT
Authors: M. Rodriguez Lopez, A. Nieto Vazquez, R. Rodriguez Nuñez, C. Albo Lopez
Journal: HemaSphere (2019)

Background: Epidemiological data in Haemophilia A (HA) report an improvement in haemophilia care over the last 50 years; the life expectancy of these patients now approaches that of the general population but evidence-based guidelines for the acute management and secondary prophylaxis of cardiovascular diseases in haemophilia patients are still lacking, Aims: We report the surgical and haematological strategies used in a mild haemophilia A patient who successfully underwent endovascular aortic aneurysm repair (EVAR) because very little is known about how to handle the increased risk of bleeding associated with invasive procedures and antithrombotic agents Methods: Case report: To our knowledge, about six other haemophilic patients treated with open surgery or EVAR were reported in the literature Results: A 64-year-old man with mild-moderate HA (F8:C 6.9%), without inhibitor was referred for treatment of a symptomatic infrarenal aortic aneurysm that had enlarged from 5.6 cm to 6 cm in diameter in 6 months. Among the personal antecedents: Acute Coronary Syndrome (1997), allergy to FVIII Concentrate of plasmatic origin, traumatic amputation of right lower limb and Arterial Hypertension on treatment.He are VIH positive on antiretroviral triple therapy. The patient was successfully treated with EVAR, Femoro-femoral bypass and right common iliac artery embolization with Coils (Endurant 32∗14 mm) associated with perioperative administration of titrated doses of recombinant full length 3rd generation factor VIII concentrate (Octocog Alfa, Advate®, Takeda Lab)(rCF8), administered on bolus The surgery was carried out under general anaesthesia and anticoagulation with bolus of unfractionated heparin(UFH)(Heparin Leo® 5000 UI) administered inmediately before the surgery and after the perioperative administration of a bolus of aproximately 55 UI by kilogram of Advate® to achieve a secure level of FVIII presurgery (105 %). 3 hours after the first bolus, an intraoperative control of FVIII was performed (using a chromogenic technique) and a new bolus of rCF8 was administered (aprox 20 UI by kilogram). At the end of surgery the UFH was reversed with protamine sulfate in a 1:1 ratio. To reduce the risk of hematoma, local dry weight and cold were applied after the withdrawal of the introducers. In the first 48 h post he received rCF8 to maintain minimum level of 80% and after, every 12 h/7 days (minimum level 50%) and another 7 days, every 24 h (minimum level 30%), performing antithrombotic prophylaxis (Enoxaparin sodium 40 mg / day) and concomitant antiaggregant therapy(Acetylsalicylic acid 100 mg / day, 2 months).The determination of the postoperative levels of FVIII was carried out by one stage coagulative assay (ACL Top). There were no perioperative complications and he was discharged at day 14, performing the determination of inhibitor at 15 days and 2 months after surgery, being negative (< 0.4 Bethesda units) Summary/Conclusion: This case report shows that EVAR is a relatively safe procedure in haemophilic patients by means of perioperative factor replacement and significantly reduce the haemorrhagic risk associated with the procedure and should be considered as first-line therapy in patients with clinically relevant haemorrhagic disorders. It́s important to report all existing experiences in order to improve the management of these situations and to develop clinical guidelines based on scientific evidence.