Percutaneous coronary intervention in a patient with factor XI deficiency

Factor XI deficiency is a rare coagulopathy occurring primarily in Ashkenazi Jews, and is associated with bleeding diathesis. This disorder complicates management during invasive procedures and poses a challenge during the performance of percutaneous coronary intervention (PCI). This situation is unique because there is a contradictory need to achieve procedural anticoagulation while minimizing the risk of bleeding. There is only one case report on the management of anticoagulation during PCI in patients with Factor XI deficiency advocating the avoidance of anticoagulation during the procedure. There is some evidence to suggest that severe Factor XI deficiency may be protective against ischemic stroke, but not against myocardial infarction. It appears, therefore, that the prolonged aPTT observed in these patients might not reflect a complete anticoagulant state and cannot be relied upon to provide procedural anticoagulation during PCI. We report our experience which suggests that PCI in these patients may be performed safely in the usual manner following the administration of plasma.