Successful Coronary Artery Bypass Grafting in a Patient with Severe Congenital Factor VII Deficiency: A Case Report

Abstract

Background: Congenital Factor VII (FVII) deficiency is a rare autosomal recessive blood-clotting disorder with a wide range of clinical presentations. Patients with severe deficiency, especially those with preoperative activity levels below 10%, face a markedly higher risk of bleeding after major surgery.

Case Presentation: We report the case of a 65-year-old man with a history of diabetes and severe FVII deficiency who was admitted with Non-ST Elevation Myocardial Infarction (NSTEMI) and underwent Coronary Artery Bypass Grafting (CABG). Perioperative hemostatic management included recombinant activated Factor VII (rFVIIa) and Fresh Frozen Plasma (FFP) due to extremely low FVII activity (<1%). Remarkably, the patient experienced an acute intraoperative myocardial infarction, likely related to FVII replacement. To our knowledge, this may be the first reported case of an intraoperative thrombotic event following rFVIIa administration during CABG for congenital FVII deficiency.

Conclusions: Despite these complications, the patient successfully had the surgery with positive outcomes, thanks to a multidisciplinary approach and careful adherence to rFVIIa dosing guidelines. This case highlights the need for tailored perioperative strategies and underscores the importance of further research to establish standardized management protocols for high-risk patients with rare bleeding disorders.