Cardiopulmonary bypass in a child with severe Factor XII deficiency

Perfusion ◽  
2021 ◽  
pp. 026765912199930
Author(s):  
Nicole Shrimpton ◽  
Aditya Patukale ◽  
Mark Rane ◽  
Pasquale Barbaro ◽  
Nelson Alphonso ◽  
...  

Factor XII (FXII) deficiency presents as a prolonged activated partial thromboplastin time (aPTT) but is not associated with clinically significant bleeding. Activated clotting time (ACT) is used routinely to monitor anticoagulation with unfractionated heparin in patients undergoing cardiopulmonary bypass (CPB). The coagulation activator reagents in most ACT tests are dependent on adequate FXII concentrations to initiate contact factor coagulation pathways. We report the case of a 14.7 kg girl undergoing CPB with a pre-admission FXII concentration of <1% and aPTT >200 seconds. The child was transfused with fresh-frozen plasma to replenish FXII, allowing safe ACT monitoring of heparin anticoagulation throughout CPB.

Perfusion ◽  
2020 ◽  
pp. 026765912094843
Author(s):  
Kazuhiro Shirozu ◽  
Yuji Karashima ◽  
Ken Yamaura

Introduction: Supplementation of fresh frozen plasma immediately after cardiopulmonary bypass is an effective method to enhance clotting ability as coagulation factors are consumed in the extracorporeal circuit during cardiopulmonary bypass. On the other hand, the anticoagulation factors in fresh frozen plasma can also deter the clotting ability. This study investigated the effect of fresh frozen plasma administration on the comprehensive clotting ability following cardiopulmonary bypass. Methods: This prospective observational study included 22 patients scheduled for cardiac surgery. Clotting times and maximum clot firmness were evaluated using the types of rotational thromboelastometry, intrinsic rotational thromboelastometry, and heparinase thromboelastography preoperatively, immediately after cardiopulmonary bypass, and 1 hour after cardiopulmonary bypass. Activated clotting time, antithrombin activity, and heparin concentration were also measured at these time-points. Results: Antithrombin activity (62.9 ± 7.2% vs. 51.1 ± 7.4%, p < 0.0001) and activated clotting time (132.6 ± 9.6% vs. 120.0 ± 9.0%, p < 0.001) were significantly higher 1 hour after cardiopulmonary bypass compared to measurements taken immediately after cardiopulmonary bypass. Heparin concentration 1 hour after cardiopulmonary bypass was significantly decreased compared to that immediately after cardiopulmonary bypass. On the other hand, maximum clot firmness determined via intrinsic rotational thromboelastometry was significantly greater 1 hour after cardiopulmonary bypass (53.8 ± 4.8 mm) than that immediately after cardiopulmonary bypass (49.5 ± 4.8 mm). Clotting time determined via intrinsic rotational thromboelastometry and heparinase thromboelastography was also significantly shorter 1 hour after cardiopulmonary bypass than that immediately after cardiopulmonary bypass. Conclusion: Fresh frozen plasma administration increased antithrombin activity and caused activated clotting time prolongation, but then increased clotting ability. Thus, testing by rotational thromboelastometry after cardiopulmonary bypass could be valuable in the detection of comprehensive clotting ability.


1996 ◽  
Vol 2 (2) ◽  
pp. 103-106 ◽  
Author(s):  
Mehraboon S. Irani

Antithrombin, a serine protease inhibitor, plays an important role in the regulation of the coagula tion cascade. It inhibits thrombin as well as factors Xa, IXa, XIa, and XIIa. Heparin markedly accelerates the rate at which antithrombin inhibits these enzymes. To prevent the formation of clots in the extracorporeal cir cuit, heparin is used during cardiopulmonary bypass sur gery (CPB). However, in a small proportion of patients, adequate anticoagulation, defined as an activated clotting time (ACT) of >400 s after an intravenous bolus of hep arin in a dose of 300 U/kg, is not achieved. Conventional treatment of heparin resistance includes fresh frozen plasma or large doses of heparin. Antithrombin concen trates were given to 12 patients who were considered to be heparin resistant at our institution. All patients had received >300 U/kg of heparin before antithrombin con centrates were infused. In all patients, the ACT pro longed to >400 s after concentrates were infused, and in nine of 12 cases to >600 s. Of these nine patients, six (67%) did not receive any allogeneic transfusions, whereas three (33%) required allogeneic blood transfu sions. In nine of 12 patients, no more heparin was given after infusing antithrombin concentrate. It appears that antithrombin concentrate, which is a fractionated, pas teurized plasma product, is a possible safer alternative to excessive heparin doses or fresh frozen plasma for CPB patients who are heparin resistant. Key Words: An tithrombin—Cardiopulmonary bypass—Heparin resis tance.


2005 ◽  
Vol 102 (2) ◽  
pp. 276-284 ◽  
Author(s):  
Michael S. Avidan ◽  
Jerrold H. Levy ◽  
Jens Scholz ◽  
Elise Delphin ◽  
Peter M. J. Rosseel ◽  
...  

Background The study evaluated the efficacy of recombinant human antithrombin (rhAT) for restoring heparin responsiveness in heparin resistant patients undergoing cardiac surgery. Methods This was a multicenter, randomized, double-blind, placebo-controlled study in heparin-resistant patients undergoing cardiac surgery with cardiopulmonary bypass. Heparin resistance was diagnosed when the activated clotting time was less than 480 s after 400 U/kg heparin. Fifty-four heparin-resistant patients were randomized. One cohort received 75 U/kg rhAT, and the other received normal saline. If the activated clotting time remained less than 480 s, this was considered treatment failure, and 2 units fresh frozen plasma was transfused. Patients were monitored for adverse events. Results Only 19% of patients in the rhAT group received fresh frozen plasma, compared with 81% of patients in the placebo group (P &lt; 0.001). During their hospitalization, 48% of patients in the rhAT group received fresh frozen plasma, compared with 85% of patients in the placebo group (P = 0.009). Patients in the placebo group required higher heparin doses (P &lt; 0.005) for anticoagulation. There was no increase in serious adverse events associated with rhAT. There was increased blood loss 12 h postoperatively (P = 0.05) with a trend toward increased 24-h bleeding in the rhAT group (P = 0.06). There was no difference between the groups in blood and platelet transfusions. Conclusion Treatment with 75 U/kg rhAT is effective in restoring heparin responsiveness and promoting therapeutic anticoagulation in the majority of heparin-resistant patients. Treating heparin-resistant patients with rhAT may decrease the requirement for heparin and fresh frozen plasma.


2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Keisuke Yoshida ◽  
Shiori Tanaka ◽  
Yuki Sato ◽  
Kazuhiro Watanabe ◽  
Kenichi Muramatsu ◽  
...  

Abstract Background This case report presents a case of a patient with global cerebral infarction of uncertain etiology following an emergency surgery for acute type A aortic dissection. As a result, factor XII deficiency was revealed postoperatively. To date, there have been several reports of cardiovascular surgery in patients with factor XII deficiency. However, all previous reports were of patients whose factor XII deficiency had been detected preoperatively; therefore, before this, there had been no reports of complications associated with factor XII deficiency following cardiovascular surgery. Case presentation We report a case of emergency aortic arch replacement surgery for acute type A aortic dissection in a 57-year-old Japanese man. A blood test prior to the surgery showed coagulopathy, a platelet count of 117 × 109/L, a prothrombin time–international normalized ratio of 1.78, an activated partial thromboplastin time of 69.7 seconds, and fibrinogen < 50 mg/dl. A smaller-than-usual dose of heparin (8000 IU) was administered because the patient’s activated clotting time was extremely prolonged (> 999 seconds). After the heparin administration, the activated clotting time, measured every 30–60 minutes, remained unchanged (> 999 seconds); therefore, additional heparin was not administered during the surgery, and there was no clinical problem during cardiopulmonary bypass. However, a diagnosis of global cerebral infarction was made on the first postoperative day. An additional blood coagulation test performed on postoperative day 9 revealed factor XII deficiency (8.0%). Regarding the reason that global cerebral infarction occurred in the present case, two reasons were considered: One was factor XII deficiency itself, and the other was low-dose heparin administration during the cardiopulmonary bypass due to excessively prolonged activated clotting time caused by factor XII deficiency. Conclusions Factor XII deficiency should be considered in patients with prolonged activated clotting time and spontaneous thrombosis in vascular surgeries. Moreover, the present case emphasizes that management of heparin during cardiopulmonary bypass should not be performed on the basis of activated clotting time monitoring alone, especially in a case with prolonged activated clotting time.


1988 ◽  
Vol 69 (2) ◽  
pp. 254-256 ◽  
Author(s):  
RAYMOND C. ROY ◽  
MICHAEL A. STAFFORD ◽  
ALLEN S. HUDSPETH ◽  
WAYNE MEREDITH

Perfusion ◽  
2020 ◽  
pp. 026765912094935
Author(s):  
Han Li ◽  
Cyril Serrick ◽  
Vivek Rao ◽  
Paul M Yip

Background: In cardiac surgery on cardiopulmonary bypass (CPB), heparin anticoagulation is monitored by point-of-care measurement of activated clotting time (ACT). The objective of this study was to compare four ACT systems in cardiac surgery in terms of their reproducibility, agreement and potential clinical impact at relevant medical decision points. Methods: The study included 40 cardiac surgery patients. Samples were taken at five time points before (T1), after heparinization for CPB (T2, T3, T4), and after heparin reversal (T5). The reproducibility, correlation, and differences in ACT values were assessed with two devices from each of the four ACT systems: Instrumentation Laboratory Hemochron Elite (Hmch), Medtronic HMS Plus (HMS), Abbott i-STAT, and Helena Abrazo. Subrange analyses were performed for low ACT values (results from T1, T5) and high ACT values (results from T2, T3, T4). Results: Within-system analysis showed strong linear correlation between paired measurements (R = 0.968-0.993). However, Hmch showed poorer reproducibility with highest proportion of values that exceed a difference of 10% and highest overall standard error of 74 seconds across the measurement range compared to that of the others (range 39-47 seconds, respectively). For inter-system comparison, using Hmch as reference, ACTs were strongly correlated as follows: HMS (R = 0.938), i-STAT (R = 0.911), and Abrazo (R = 0.911). Agreement analysis in the high ACT range showed HMS tended to have higher ACT values with +11% bias over Hmch, whereas i-STAT (–8% bias) and Abrazo (–13% bias) tended to underestimate. Post-protamine ACT results were dependent on device type where Hmch yielded highest post-protamine ACT (+13% higher than baseline) compared to –16% for HMS, –10% for iSTAT and 0% for Abrazo. Conclusions: Each device had individual reproducibility and biases, which may impact peri-operative heparin management. Careful validation must be undertaken when adopting a different method as decision limits would be affected. Clinicians should also be cautious using ACT as the only indicator for full heparin reversal.


1985 ◽  
Vol 63 (Supplement) ◽  
pp. A40 ◽  
Author(s):  
R. E. Barnette ◽  
R. C. Shupak ◽  
W. R. Shepard ◽  
A. Koneti Rao

2013 ◽  
Vol 119 (4) ◽  
pp. 1050-1057 ◽  
Author(s):  
Marie Roguski ◽  
Kyle Wu ◽  
Ron I. Riesenburger ◽  
Julian K. Wu

Object A primary goal in the treatment of patients with warfarin-associated subdural hematoma (SDH) is reversal of coagulopathy with fresh-frozen plasma. Achieving the traditional target international normalized ratio (INR) of 1.3 is often difficult and may expose patients to risks of volume overload and of thromboembolic complications. This retrospective study evaluates the risk of mild elevations of INR from 1.31 to 1.69 at 24 hours after admission in patients presenting with warfarin-associated SDH. Methods Sixty-nine patients with warfarin-associated SDH and 197 patients with non–warfarin-associated SDH treated at a single institution between January 2005 and January 2012 were retrospectively identified. Charts were reviewed for patient age, history of trauma, associated injuries, neurological status at presentation, size and chronicity of SDH, associated midline shift, INR at admission and at hospital Day 1 (HD1), concomitant aspirin or Plavix use, platelet count, and medical comorbidities. Patients were stratified according to use of warfarin and by INR at HD1 (INR 0.8–1.3, 1.31–1.69, 1.7–1.99, and ≥ 2). The groups were evaluated for differences the in rate of radiographic expansion of SDH and in the rate of clinically significant SDH expansion resulting in death, unplanned procedure, and/or readmission. Results There was no difference in the rate of radiographic versus clinically significant expansion of SDH between patients not on warfarin and those on warfarin (no warfarin: 22.3% vs 20.3%, p = 0.866; warfarin: 10.7% vs 11.6%, p = 0.825), but the rate of medical complications was significantly higher in the warfarin subgroup (13.3% for patients who did not receive warfarin vs 26.1% for those who did; p = 0.023). For warfarin-associated SDH, there was no difference in the rate of radiographic versus clinically significant expansion between patients reversed to HD1 INRs of 0.8–1.3 and 1.31–1.69 (HD1 INR 0.8–1.3: 22.5% vs 20%, p = 1; HD1 INR 1.31–1.69: 15% vs 10%, p = 0.71). Conclusions Mild INR elevations of 1.31–1.69 in warfarin-associated SDH are not associated with a markedly increased risk of radiographic or clinically significant expansion of SDH. Larger prospective studies are needed to determine if subtherapeutic INR elevations at HD1 are associated with smaller increases in risk of SDH expansion.


Sign in / Sign up

Export Citation Format

Share Document