|Year : 2019 | Volume
| Issue : 3 | Page : 97-99
Coronary artery stenting in a patient with factor XI deficiency
Mingqiang Fu, Chenguang Li, Shufu Chang, Jianying Ma, Junbo Ge
Department of Cardiology, Zhongshan Hospital, Fudan University, Shanghai Institute of Cardiovascular Diseases, Shanghai, China
|Date of Submission||16-Apr-2019|
|Date of Acceptance||24-Jul-2019|
|Date of Web Publication||30-Sep-2019|
Department of Cardiology, Zhongshan Hospital, Fudan University, Shanghai Institute of Cardiovascular Diseases, Shanghai
Source of Support: None, Conflict of Interest: None
Factor XI deficiency is a rare coagulopathy which has a higher risk of bleeding following trauma or an operation. Stable coronary artery disease patients complicated by factor XI deficiency draw extraordinary caution when coronary artery stents are needed, for interventional cardiologists must balance the need for antithrombosis and bleeding complications. Currently, there is no guideline for the management of such patients. Here, we describe a patient with coronary artery disease with factor XI deficiency who underwent an uneventful percutaneous coronary intervention.
Keywords: Bleeding, coronary artery disease, factor XI deficiency, percutaneous coronary intervention
|How to cite this article:|
Fu M, Li C, Chang S, Ma J, Ge J. Coronary artery stenting in a patient with factor XI deficiency. Cardiol Plus 2019;4:97-9
| Introduction|| |
Factor XI deficiency is an autosomal transmitted coagulopathy which is particularly common among Ashkenazi Jews at a heterozygote frequency of 8%–10%., However, this disease is quite rare in other ethnic groups as the assumed incidence in general population is one in a million persons. Patients of factor XI deficiency have a higher risk of bleeding, which mainly occurs following trauma or surgery, especially in tissues with a high local fibrinolytic activity. However, clinical presentation of factor XI-deficient patients varies from the absence of symptoms to life-threatening bleeding. When such patients need to undergo percutaneous coronary interventions (PCIs), there is a delicate balance between serious complications due to bleeding and prevention of thrombosis. Here, we present a case of a patient with coronary artery disease complicated by factor XI deficiency undergoing successful PCI.
| Case Report|| |
A 58-year-old male (172 cm in height and 62 kg in weight) was admitted to our hospital for the treatment of worsening effort angina. He had a history of hypertension and hyperlipidemia for over 10 years and smoked a package of cigarettes per day over 20 years. When admitted, the patient reported that he always experienced prolonged bleeding and was diagnosed with factor XI deficiency 3 years prior in another local hospital. There was no history of injury-associated bleeding and no family history existed. Preoperative blood tests revealed an elevated activated partial thromboplastin time at 91 s (range: 25–31.3 s). Complete blood count with platelet count, thrombin time, and prothrombin time was normal. Further analysis revealed that factor XI activity was 2.1% of control (normal range: 50%–150%) which was considered severe factor XI deficiency.
After a loading dose of aspirin 300 mg and clopidogrel 300 mg, coronary angiography was performed on the patient by radial artery approach which revealed severe stenotic lesions at the middle of left anterior descending coronary artery (LAD), a drug-eluting stent was then implanted in target lesion [Figure 1] with 5000 U of unfractionated heparin, and activated clotting time of whole blood was 285 s (range: 60–130 s) in the operation. The patient was subsequently prescribed aspirin 100 mg/d and clopidogrel 75 mg/d as a dual antiplatelet therapy postoperation. He had no problems with bleeding or bruising either in the immediate perioperative period or during the regular outpatient visits for 6 months.
|Figure 1: Coronary angiography and percutaneous coronary intervention to the severe stenotic lesion at the middle of left anterior descending coronary artery (LAD). Drug-eluting stent (Promus PREMIER 3.0 mm × 38 mm) was successfully deployed. CRA: Cranial projection, RAO: Right anterior oblique projection, CAU: Caudal projection|
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| Discussion|| |
Coagulation factor XI is a plasma glycoprotein that participates in the early phase of the intrinsic pathway for secondary hemostasis, and it is essential for normal hemostasis. Another important function of factor XI activation is to reduce fibrinolysis by promoting activation of thrombin-activatable fibrinolysis inhibitor. Hereditary factor XI deficiency was first reported in 1953 as a “hemophilia-like disease” manifested as a mild bleeding diathesis. Patients with factor XI deficiency may experience delayed postoperative bleeding, but spontaneous bleeding is rare. Furthermore, the bleeding tendency is unpredictable, and the CRUSADE bleeding risk score is inapplicable in such patients. Thus, clinicians are sometimes unaware of the presence of this disorder in patients without an obvious bleeding history. A prolonged activated partial thromboplastin time may be the initial presentation in an asymptomatic patient. Low factor XI levels in the absence of a circulating factor XI inhibitor confirm the diagnosis.
For interventional cardiologists, this disorder may cause unique problems during PCI, especially when coronary artery stents are used. In cases of cardiac intervention, this is followed by heparin infusion to decrease the thrombotic danger associated with the procedure; meanwhile, antiplatelet drugs after intervention also increase the risk of bleeding. Because patients with factor XI deficiency are at particular risk of bleeding complications, cardiologists must be caution to balance the need for antithrombosis and minimizing related bleeding complications.
In this case, to minimize the potential bleeding risk, we took the radial artery approach rather than the femoral artery approach. Furthermore, activated clotting time was monitored during the procedure. There is no guideline or expert consensus for the management of cardiac intervention in patients with factor XI deficiency due to the scarcity of cases. An exhaustive literature search resulted in three published cases. Lee et al. reported a 52-year-old male with unstable angina who underwent uneventful PCI by femoral artery approach. They used fresh frozen plasma transfusion prior to the procedure, followed by intravenous heparin and a close monitoring of the activated clotting time during catheterization. Mungee et al. reported a complicated case of a 72-year-old male with a positive stress test who underwent a successful PCI by femoral artery approach. The patient had a history of multiple episodes of severe bleeding and fresh frozen plasma transfusions, and then, he was given factor XI inhibitor prior to catheterization. Despite the lack of heparinization, the patient experienced an uneventful, nonthrombotic outcome. Kaku et al. described an 84-year-old female with worsening effort angina and an unknown history of factor XI deficiency who underwent a PCI. The patient's factor XI deficiency diagnosis was suspected because of high activated clotting time during the first PCI. Interestingly, a second PCI by radial artery approach with a favorable outcome was done within the same admission without the use of fresh frozen plasma nor heparin.
To summarize the reported cases, it appears that factor XI deficiency might not reflect a complete coagulant state in stable coronary artery disease patients, and PCI may be performed safely in the usual manner.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
This work was supported by grants from the National Natural Science Foundation of China (81700225).
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Duga S, Salomon O. Congenital factor XI deficiency: An update. Semin Thromb Hemost 2013;39:621-31.
Shearin-Patterson T, Davidson E. Hemophilia C. JAAPA 2013;26:50.
Girolami A, Ferrari S, Sambado L, Peroni E, Cosi E. Myocardial infarctions and other acute coronary syndromes in rare congenital bleeding disorders: A critical analysis of all reported cases. Clin Appl Thromb Hemost 2015;21:359-64.
Salomon O, Steinberg DM, Seligshon U. Variable bleeding manifestations characterize different types of surgery in patients with severe factor XI deficiency enabling parsimonious use of replacement therapy. Haemophilia 2006;12:490-3.
Bolton-Maggs PH. Factor XI deficiency resolving the enigma? Hematology Am Soc Hematol Educ Program 2009:97-105.
Von dem Borne PA, Bajzar L, Meijers JC, Nesheim ME, Bouma BN. Thrombin-mediated activation of factor XI results in a thrombin-activatable fibrinolysis inhibitor-dependent inhibition of fibrinolysis. J Clin Invest 1997;99:2323-7.
Rosenthal RL, Dreskin OH, Rosenthal N. New hemophilia-like disease caused by deficiency of a third plasma thromboplastin factor. Proc Soc Exp Biol Med 1953;82:171-4.
Wheeler AP, Gailani D. Why factor XI deficiency is a clinical concern. Expert Rev Hematol 2016;9:629-37.
Lee S, Jeong M, Sohn I, Lim S, Hong S, Kang D, et al
. Successful management of a patient with factor XI deficiency and unstable angina by percutaneous coronary intervention. Korean Circ J 2005;35:860-3.
Mungee S, Lapin R, Cavusoglu E, Clark LT, Marmur JD. Percutaneous coronary intervention in a patient with congenital factor XI deficiency and acquired inhibitor. Cardiology 2007;107:69-72.
Kaku B, Shimojima M, Yoshida T, Katsuda S, Taguchi T, Nitta Y, et al.
Coronary artery stenting in a patient with factor XI deficiency. Cardiovasc Interv Ther 2012;27:99-104.