Continuous infusion of factor VIIIc during heart surgery in a patient with haemophilia A

Bels, David De; Demeere, J.-L.; Dugauquier, J; Louis, Patrick J.; Vorst, E. Van der

doi:10.1097/00003643-200412000-00011

Cited by 13 publications

(12 citation statements)

References 5 publications

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“…Similarly, De Bels et al [15] performed successful CABG and MVR (mechanical) on a 53-year-old male with haemophilia A using factor VIII concentrate infusion.…”

Section: Resultsmentioning

confidence: 99%

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Rossi

Jayaram

Sayeed

2011

Interactive CardioVascular and Thoracic Surgery

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A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether patients with haemophilia undergoing cardiac surgery have good surgical outcomes. Haemophilia A and haemophilia B are sex-linked recessive inherited diseases affecting males only, with females acting as carriers. The conditions result in various degrees of factor VIII or factor IX deficiency, respectively. The life expectancy of haemophilia patients is increasing and now approaches that of the general male population, and they are confronted with age-related co-morbidity, including ischaemic cardiovascular disease. Replacement of the deficient factor (VIII for haemophilia A and IX for haemophilia B) is the cornerstone of treatment; other therapeutic options include tranexamic acid, desmopressin and aprotinin. Recently, the advent of recombinant factor VIII and IX has eliminated the infective risk of using factor concentrates, such as prothrombin complex concentrate or fresh frozen plasma. A total of 84 papers were found using the reported search criteria, and out of this 25 papers, selected with reference to a more modern date range, provided the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results were tabulated. We conclude that there is lack of good-quality evidence and that, in all probability, these papers are subject to publication bias as poor outcomes are unlikely to have been reported. However, all the reported series showed that good outcomes are possible in this specific subgroup of patients given the correct approach. The data accrued from these studies (a total of 30 adults and three children) suggest that routine cardiac surgery can be performed safely in patients with haemophilia, with minimal morbidity and mortality. We identified the following key points to achieve this result: a team approach, a factor replacement protocol and perioperative monitoring of factor levels. Intraoperative plasma factor levels can be easily measured before heparin and after protamine sulphate administration, whereas during cardiopulmonary bypass this will require a chromogenic method. Exposure to factor concentrates early in the life might predispose a patient with severe haemophilia to the development of inhibitors. Moreover, the absence of inhibitors should be confirmed before any surgical procedure.

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“…Similarly, De Bels et al [15] performed successful CABG and MVR (mechanical) on a 53-year-old male with haemophilia A using factor VIII concentrate infusion.…”

Section: Resultsmentioning

confidence: 99%

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Rossi

Jayaram

Sayeed

2011

Interactive CardioVascular and Thoracic Surgery

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“…In most cases, pre-operatively FVIII or FIX levels were targeted to be more than 80% of normal and this was achieved with bolus infusion of either a plasma-derived 5,11,16,17,21 or recombinant factor concentrate. 3,5,8,10,13,14,19 Continuous infusion has rarely been given pre-operatively.…”

Section: Review Of the Literaturementioning

confidence: 99%

“…During CABG the antifibrinolytics that were given were aminocaproid acid, 9,19,22 aprotinin, 11,21 and trans-4-aminomethylcyclohexanecarboxylic acid (tranexamid acid). 10,13,17 Desmopressin (DDAVP) was given in one patient with mild hemophilia A. 20 Another critical tool to reduce the number of allogeneic transfusions was the use of cell-saver systems for autologous blood recovery.…”

Section: Review Of the Literaturementioning

confidence: 99%

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Replacement therapy for coronary artery bypass surgery in patients with hemophilia A and B

Kanellopoulou

Nomikou

2018

J Card Surg

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There are only a few cases of patients with hemophilia A and B who have undergone coronary artery bypass surgery. High levels of replacement therapy with factor concentrate either with bolus or continuous infusion are usually required pre-operatively and during the first post-operative days in order to maintain the coagulation deficient factor levels greater than 80% of normal. Heparinization during cardiopulmonary bypass appears to be safe and intra-operative blood salvage using cell saver techniques reduce the need for transfusions.

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“…Menorrhagia in women with bleeding disorders often is successfully managed with antifibrinolytics and hormone therapy, 85 including combination or progestin-only contraceptives for maintenance ther- Interfere with the formation of the fibrinolytic enzyme plasmin from its precursor plasminogen First-line therapy in rare disorders of fibrinolysis (␣2-antiplasmin and PAI-1 deficiencies) 56 Systemic or topical antifibrinolytics may be used alone to treat or prevent mucosal bleeding in patients with coagulation factor disorders, thus avoiding systemic hemostatic therapies 1,[66][67][68]73 Effective, first-line therapy for menorrhagia (with or without hormonal therapies) 25,80,85 Tranexamic acid used anecdotally in patients with bleeding disorders, including as an adjunct to factor replacement for major surgery in patients with hemophilia 78,81,84 and as a sole systemic hemostatic agent primarily for minor procedures 1,66 Also used for treatment of bleeding and for hemostatic coverage for minor surgery in patients with platelet function disorders; may be used adjunctively with platelet transfusion and other nontransfusional therapies in these patients 83 Theoretical risk for thrombogenesis; may be increased in patients who receive concurrent factor replacement or who have certain bleeding disorders (eg, dysfibrinogenemia, in which antifibrinolytics are contraindicated)…”

Section: Nontransfusional Hemostatic Therapiesmentioning

confidence: 99%

Identification and Basic Management of Bleeding Disorders in Adults

Kruse‐Jarres

Singleton

Leissinger

2014

The Journal of the American Board of Family Medicine

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Adults with bleeding disorders may present to their family physician with minor bleeding symptoms or hematologic laboratory abnormalities discovered during evaluation for surgery or another purpose. Identifying the small proportion of adults who have an underlying bleeding disorder as the cause for such signs or symptoms may be challenging. In cases of asymptomatic hematologic laboratory abnormalities, the particular abnormality should narrow down the potentially affected hemostatic component(s), ideally streamlining subsequent investigation. In patients presenting with bleeding symptoms, a thorough history and physical examination are critical for first identifying bleeding as pathologic, then performing the appropriate diagnostic evaluation after excluding identifiable causes. Knowledge of the pathophysiologic processes contributing to impaired hemostasis in any given bleeding disorder ensures proper treatment and avoids therapies that are unnecessary or even contraindicated. Management is further determined by bleeding phenotype and, for invasive procedures, the anticipated risk for bleeding. Consultation with a hematologist may facilitate proper evaluation and treatment, particularly in adults with rare bleeding disorders or no identifiable cause for bleeding. This article reviews the diagnostic approach to hematologic laboratory abnormalities and abnormal bleeding in adults, as well as basic preventive care and hemostatic management of adults with bleeding disorders. (J Am Board Fam Med 2014;27:549 -564.)

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Continuous infusion of factor VIIIc during heart surgery in a patient with haemophilia A

Cited by 13 publications

References 5 publications

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Replacement therapy for coronary artery bypass surgery in patients with hemophilia A and B

Identification and Basic Management of Bleeding Disorders in Adults

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