Background Patients with acquired hemophilia A (AHA) who require open heart surgery have a life-threatening risk of hemorrhage. Limited data exist to guide perioperative management of these patients. Case presentation A 53-year-old female with rheumatoid arthritis, concomitant aortic valve endocarditis, and severe aortic regurgitation presented to our hospital. Bleeding and abnormal coagulation tests were noted during the initial workup, and she was diagnosed with AHA. The perioperative management plan included the use of pharmaceuticals, porcine recombinant factor VIII, and blood products. Extensive preoperative coagulation data were obtained, and factor VIII levels were continuously monitored to mitigate bleeding complications. The aortic valve replacement and root repair were uneventful. Conclusion Cardiac surgery in patients with AHA is possible as long as complex perioperative hemostatic and hematology management is used.

Perioperative bleeding can be a serious life-threatening complication in adult patients undergoing cardiac surgery, given the older age and additional comorbidities present in this patient population. The standard treatment options include transfusion of blood components and surgical re-exploration. We report the first case of an elderly female patient treated with local administration of recombinant factor VIIa (rFVIIa) for intractable hemorrhagic pericardial effusion, which developed following a transcutaneous aortic valve replacement (TAVR) procedure for severe aortic stenosis. No thromboembolic phenomena or adverse effects were observed. Local administration of rFVIIa is an efficacious treatment option for cardiac surgery patients as opposed to systemic administration of rFVIIa, use of massive blood products, or surgical re-exploration.

Remote ischemic preconditioningfor renal protection in children undergoing surgery for complex congenital heart disease: What do we know now and where do we go next?

Context.-Proper diagnosis and therapy of fibrinogen deficiency requires high-quality fibrinogen assays.Objective.-To assess the interlaboratory bias, precision, and grading of fibrinogen assays used by laboratories participating in the United States College of American Pathologists proficiency testing program in coagulation.Design.-Two identical vials of normal plasma were sent to more than 3500 laboratories. Participants measured fibrinogen levels using local methods.Results.-Fifty different fibrinogen methods were evaluated. All-method bias was 8.3% (range of method-specific biases, 0.0%-27.0%) and all-method coefficient of variation was 7.7%(range of method-specific coefficients of variation, 0.7%-25.8%). After controlling for reagent/instrument type, mean fibrinogen levels were 11.6% higher for prothrombin time-based reagents compared to Clauss (P<.001), and coefficient of variation was 46% lower for mechanical endpoint instruments compared to photo-optical. Most testing events (97.4%) could be reliably graded as pass or fail using a target range of +/- 20% from the method mean (total pass rate, 98.8%). Total fail rate was 3.0-fold lower for mechanical instruments compared to photo-optical (0.5% versus 1.5%, P=.001). Nonetheless many photo-optical methods had very high precision and very low fail rates.Conclusions.-Fibrinogen assays showed highly variable methodology and performance characteristics. Bias, precision, and grading were affected by the type of reagent or instrument used. (Arch Pathol Lab Med. 2012; 136: 789-795; doi: 10.5858/arpa.2011-0322-OA)

CONTEXT Hereditary and acquired deficiencies of antithrombin (AT), protein C (PC), and protein S (PS) are risk factors for venous thromboembolism. Proper diagnosis requires high-quality assays for these proteins. OBJECTIVE To determine the accuracy and interlaboratory precision of AT, PC, and PS assays used by laboratories participating in the United States College of American Pathologists proficiency testing program in thrombophilia and to grade the performance of laboratories. DESIGN Standardized normal plasma with assigned analyte values was sent in 2 separate challenges to participating laboratories. Participants measured AT, PC, and PS levels using local methods. RESULTS When compared with the assigned values for the international standard, the order of assay accuracy from highest to lowest was AT activity, PC antigen, AT antigen, total PS antigen, PC activity, PS activity, and free PS antigen (range of assay bias, 2.6%-8.8%). The order of assay precision from highest to lowest was PC activity, AT activity, AT antigen, total PS antigen, PS activity, free PS antigen, and PC antigen (range of assay coefficient of variation, 6.1%-20.0%). Most testing events (87.8%) could be graded as pass or fail using a target range of ±3 standard deviations from the method-specific mean. The pass rate was 98.2% for all AT, PC, and PS testing events combined. CONCLUSIONS Accuracy and precision were higher for AT assays and lower for PC and PS assays. It was feasible to grade individual laboratory performance.

TransfusionVolume 51, Issue 10 p. 2066-2077 How do we manage patients treated with antithrombotic therapy in the perioperative interval Arthur W. Bracey, Corresponding Author Arthur W. Bracey From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Arthur W. Bracey, MD, PO Box 20345, MC 4-265, Houston, TX 77225-0345; e-mail: [email protected]. Search for more papers by this authorMeredith A. Reyes, Meredith A. Reyes From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Search for more papers by this authorAlice J. Chen, Alice J. Chen From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Search for more papers by this authorMaryam Bayat, Maryam Bayat From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Search for more papers by this authorPaul M. Allison, Paul M. Allison From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Search for more papers by this author Arthur W. Bracey, Corresponding Author Arthur W. Bracey From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Arthur W. Bracey, MD, PO Box 20345, MC 4-265, Houston, TX 77225-0345; e-mail: [email protected]. Search for more papers by this authorMeredith A. Reyes, Meredith A. Reyes From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Search for more papers by this authorAlice J. Chen, Alice J. Chen From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Search for more papers by this authorMaryam Bayat, Maryam Bayat From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Search for more papers by this authorPaul M. Allison, Paul M. Allison From the Department of Pathology, Texas Heart Institute at St Luke's Episcopal Hospital, and the Department of Pharmacy, St Luke's Episcopal Hospital, Houston, Texas.Search for more papers by this author First published: 22 April 2011 https://doi.org/10.1111/j.1537-2995.2011.03146.xCitations: 6Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume51, Issue10October 2011Pages 2066-2077 RelatedInformation

Background: Changes in postoperative serum creatinine levels have been used to define acute renal injury in patients undergoing cardiac surgery with cardiopulmonary bypass. It remains unclear, however, whether subclinical increases in serum creatinine that do not meet current Acute Kidney Injury Network or RIFLE (risk, injury, failure, loss, and end-stage kidney disease) criteria for acute renal injury are predictive of mortality after cardiac surgery.Methods: Multivariate logistic regression was performed in a retrospective cohort of 3914 consecutive patients undergoing primary, isolated coronary artery bypass grafting with cardiopulmonary bypass to determine whether postoperative serum creatinine change independently predicts 30-day all-cause mortality in patients with normal renal function and with varying levels of preoperative renal insufficiency. To control further for selection bias, multivariate logistic regression was performed on a propensity-matched cohort (n = 2042) to determine whether subclinical increases in serum creatinine predict mortality.Results: Negative change in serum creatinine was associated with reduced 30-day all-cause mortality. Even subclinical increases in serum creatinine were associated with increased mortality relative to patients with negative changes in serum creatinine (odds ratio, 3.93; 95% confidence interval, 1.68-9.22; P <.01). After propensity matching, subclinical increases in serum creatinine were still associated with increased mortality (odds ratio, 4.13; 95% confidence interval, 1.37-12.45; P = .01).Conclusions: Subclinical increases in serum creatinine that do not meet acute renal injury criteria are independently associated with 30-day all-cause mortality in patients with normal renal function or preoperative renal insufficiency undergoing coronary artery bypass grafting. (J Thorac Cardiovasc Surg 2012;143:682-8)

Flow cytometry and Luminex-based single HLA antigen assays are more sensitive methods to determine the presence of HLA antibodies (Ab), donor specifically directed HLA Abs (DSA) and pre-transplant (Tx) crossmatch (FCXM) outcomes for potential heart Tx (HTX) recipients (recips). We retrospectively evaluated the effect of sensitization from pre-Tx blood transfusions (BT) received before HTx on panel reactive HLA Ab (PRA), DSA, FCXM results and post-HTx clinical outcomes.

Previous studies suggest that serum hepatocyte growth factor (HGF) level may be a useful diagnostic and prognostic biomarker for various tumors. We investigated the utility of plasma HGF level measurements in diagnosing periampullary cancer (PAC).

mmHg.The multi-step method suppressed increases in intrahepatic pressure and microbubbles.The sizes of the RFA-induced areas were not significantly different between the single-step and multi-step methods.[Conclusions] The present results demonstrate that increased intrahepatic pressure causes microbubbles, thus suggesting that some risk of dissemination due to RFA exists.Therefore, RFA should be performed while adjusting intrahepatic pressure to avoid generating microbubbles.From this perspective, the multistep LeVeen method is suitable for treatment.

Background: Dosing of recombinant activated factor VII (rFVIIa) is controversial and Unstandardized, and there is growing concern about thromboembolic complications, especially in left ventricular assist device (LVAD)-supported patients. We reviewed our experience with rFVIIa administration in patients with LVADs and examined its effectiveness and adverse effects, including the incidence of thromboembolic events and its correlation with increasing doses.Methods: We retrospectively reviewed the records of 62 patients who received an LVAD and rFVIIa at our center between January 2004 and November 2006. Patients who received a single dose of 10 to 20 mu g/kg (n = 32; 52%) constituted the "low-dose" cohort, and patients who received 30 to 70 mu g/kg (n = 30; 48%) constituted the "high-dose" cohort. Laboratory values obtained before and after rFVIIa administration, as well as patients' transfusion requirements, were compared to determine the effectiveness of rFVIIa in reversing coagulopathy and reducing blood loss. We also compared the incidence of thromboembolic events in the low- and high-dose groups.Results: Administration of rFVIIa was associated with significant decreases in prothrombin time, activated partial thromboplastin time and transfusion requirements. This association was seen in both the low- and high-dose groups. In addition, the incidence of thromboembolic events was significantly higher in the high-dose group (36.7%) than in the low-dose group (9.4%) (p <= 0.001).Conclusions: Although rFVIIa administration seemed helpful in controlling life-threatening hemorrhage, patients requiring higher doses (30 to 70 mu g/kg) had a dramatically higher incidence of serious thromboembolic events. J Heart Lung Transplant 2009;28:785-90. Copyright (C) 2009 by the International Society for Heart and Lung Transplantation.

Background: Heparin induced thrombocytopenia (HIT) is a challenging diagnosis; especially post cardiac surgery and patients with cardiac support devices. The diagnosis is based on clinicopathologic findings including pertinent clinical history, and heavy reliance on laboratory testing (Heparin PF-4 ELISA) to detect antibodies immunologically or by functional means (serotonin release assay - SRA). Experience with routine measurement of SRA in combination with HPF4 in this setting is limited.

Autoantibodies to prothrombin, first described almost 50 years ago, are paradoxically associated with thrombosis. Described is an unusual case of fatal hypercoagulability in a patient with multiple arterial and venous thromboembolic complications despite intense anticoagulation while being bridged to transplantation with a left ventricular assist device. Serum analysis revealed the presence of prothrombin autoantibodies and high titers of anti-nuclear antibodies, and autopsy revealed pulmonary arteriolar vasculitis. These findings suggest an autoimmune basis for the presence of anti-prothrombin antibodies and the hypercoagulable state observed in the present case.

West Nile virus is a mosquito-borne RNA Flavivirus infection transmitted to humans and other vertebrates, mainly by the Culex species of mosquito. Since the mid-1990s, the frequency and apparent clinical severity of West Nile virus outbreaks have increased. We report the case of a patient who developed West Nile virus encephalitis shortly after undergoing cardiac transplantation. Clinicians should be aware of the possibility of West Nile virus infection in transplant recipients and in patients receiving blood transfusions. (c) 2005 by the International Society for Heart and Lung Transplantation.

The objective of this study was to examine the pharmacokinetics of intravenous dalteparin (Fragmin, Pharmacia-Upjohn, Peapack, NJ) and to assess the accuracy of standard coagulation-based monitoring techniques as an estimate of drug concentration with which to guide dosing. Knowledge of the kinetic behavior of low-molecular-weight heparins (LMWHs) and the possible utility of coagulation times for monitoring may aid in the development of safe and effective dosing algorithms for percutaneous coronary interventions. Twenty normal volunteers were treated at 2-week intervals with each of three intravenous dalteparin doses. Measurement of anti-IIa, anti-Xa, activated partial thromboplastin time (aPTT), activated clotting time (ACT), and low-range ACT was performed at baseline and at seven additional time points over 8 hours. The half-life of intravenous dalteparin is 77 minutes with slight dose-related variation. The aPTT, LR-ACT, and standard ACT are prolonged after dalteparin administration with the increase closely correlated to anti-Xa activity (aPTT, r = 0.85; LR-ACT, r = 0.79). Classification of anticoagulation intensity range using aPTT or LR-ACT in comparison to anti-Xa activity (0.5-0.99, 1.0-1.49, 1.5-2, >2) displays a level of agreement (kappa: aPTT = 0.69, LR-ACT = 0.59) that is comparable to values reported for coagulation time guidance of unfractionated heparin administration. Standard coagulation times are sensitive to the anticoagulant effect of dalteparin with a degree of correlation that suggests their utility for estimating drug concentration during high dose therapy. Trials establishing a relationship between monitoring and clinical efficacy, and the risk/reward of different treatment ranges alone or in combination with GPIIb/IIIa inhibitors and clopidogrel, are necessary.

Background: Heparin-induced thrombocytopenia type II (HITS II) is a life threatening side effect of heparin therapy. We present data on the thrombotic consequences of developing HITS for patients supported on an axial flow left ventricular assist device (LVAD).