Total Arch Replacement for Acute Aortic Dissection (Stanford A) in a Patient With Hemophilia A

Furutachi, Akira; Furukawa, Kojiro; Oda, Yoshimasa; Morita, Shigeki

doi:10.1016/j.athoracsur.2011.10.028

Cited by 7 publications

(6 citation statements)

References 6 publications

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“…25 Multiple bolus injections of FVIII were also needed in another patient who underwent a total aortic arch replacement under deep hypothermic circulatory arrest using CIT. 26…”

Section: Discussionmentioning

confidence: 99%

“…25 Multiple bolus injections of FVIII were also needed in another patient who underwent a total aortic arch replacement under deep hypothermic circulatory arrest using CIT. 26 Patients with FVIII and FIX deficiency may demonstrate impairment in clot stability in addition to impaired clot generation. This may be due to the production of fibrin that may be more sensitive to fibrinolysis and also diminished inhibition of thrombolysis by thrombin.…”

Section: Prophylactic Factor Replacement For Hemophilia Amentioning

confidence: 99%

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Perioperative Coagulation Management of a Hemophilia A Patient During Cardiac Surgery

Odonkor

Srinivas

Strauss

et al. 2017

Semin Cardiothorac Vasc Anesth

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Perioperative management of cardiovascular surgical procedures requiring cardiopulmonary bypass (CPB) in patients with hemophilia A poses a clinical challenge in coagulation management. Use of CPB requires the administration of an anticoagulant, usually unfractionated heparin, and also causes dilutional coagulopathy, platelet dysfunction or platelet consumption coagulopathy. Hypothermia and activation of the inflammatory cascade also affect coagulation. The effects of CPB on circulating levels of factor VIII have not been clearly defined. In this review, the effects of CPB and hemodilution on FVIII are shown in a case presentation, and perioperative laboratory testing in patients with hemophilia A having cardiac surgery is discussed along with perioperative and postoperative coagulation management.

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“…25 Multiple bolus injections of FVIII were also needed in another patient who underwent a total aortic arch replacement under deep hypothermic circulatory arrest using CIT. 26…”

Section: Discussionmentioning

confidence: 99%

Section: Prophylactic Factor Replacement For Hemophilia Amentioning

confidence: 99%

Perioperative Coagulation Management of a Hemophilia A Patient During Cardiac Surgery

Odonkor

Srinivas

Strauss

et al. 2017

Semin Cardiothorac Vasc Anesth

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“…Literature reports describe the surgical management of aortic dissection in PWH. 100 , 116 , 127 , 137 Because emergent intervention is necessary, the management of acute aortic dissection poses challenges beyond those of elective cardiovascular procedures in PWH. Surgical and other considerations are summarized in a recently published first-ever case report describing management of an acute type A dissection in an individual with hemophilia A.…”

Section: Cardiovascular Conditions Requiring Operations In People Witmentioning

confidence: 99%

Consensus Review of the Treatment of Cardiovascular Disease in People With Hemophilia A and B

Ferraris

Boral

Cohen

et al. 2015

Cardiology in Review

104

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With advances in care, increasing numbers of people with hemophilia (PWH) achieve near-normal life expectancies and present with typical age-related cardiovascular conditions. Evidence-based guidelines for medical or surgical management of cardiovascular conditions in individuals with hemophilia are limited. Published recommendations exist for the management of some common cardiovascular conditions (eg, ischemic heart disease, atrial fibrillation), but identifying optimal strategies for anticoagulant or antithrombotic therapy constitutes the primary challenge of managing nonoperative cardiovascular disease (CVD) in PWH. In general, as long as factor concentrates or other hemostatic therapies maintain adequate hemostasis, the recommended medical and surgical management of CVD in PWH parallels that in individuals without hemophilia. The presence of factor inhibitors complicates hemophilia management. Published outcomes of CVD treatment in PWH are similar to those in the general population. Specific knowledge about factor replacement, factor inhibitors, and disease-specific treatment distinguishes the cardiovascular care of PWH from similar care of individuals without this rare bleeding disorder. Furthermore, a multidisciplinary approach incorporating a hematologist with an onsite coagulation laboratory, ideally associated with a hemophilia treatment center, is integral to the management of CVD in PWH.

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“…Continous factor VIII infusions were given from the time of skin incision (higher dose) until the patient was discharged (lower dose during the last few days). This enabled the patient to avoid the need for revision and to avoid receiving large numbers of blood products, as was described in previous reports [3,4].…”

Section: Commentmentioning

confidence: 75%

“…For example, in the report of a severe hemophilia A patient undergoing ascending aorta replacement caused by aortic dissection, 10 units of packed RBCs, 18 units of platelets, and 11 units of FFP were given [3]. In the case described by Furutachi and colleagues [4], a patient with mild hemophilia A who had a Stanford A aortic dissection underwent ascending aorta and aortic arch replacement and received 32 units of packed RBCs, 40 units of platelets, and 50 units of FFP. In these two cases, a lower dose of factor VIII was used, and factor VIII activity levels were maintained at approximately 100% of normal levels.…”

Section: Commentmentioning

confidence: 99%

Bentall Operation in a Patient With Severe Hemophilia A and Marfan Syndrome by Use of a Biologic Composite Graft

Yıldırım

Özbakkaloğlu

Öztürk

et al. 2016

The Annals of Thoracic Surgery

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We describe a patient with severe hemophilia A and Marfan syndrome who underwent an elective Bentall operation. Because of the severe hemophilia, anticoagulation could not be given postoperatively; thus, a biologic Valsalva conduit graft was used. During the procedure, factor VIII was given as a bolus dose just before incision, then by continous infusion intraoperatively to maintain the factor VIII activity level between 200% and 300%. Minimal postoperative bleeding occurred. The infusion was continued postoperatively at a lower dose until all chest tubes, pacing wires, and invasive catheters were removed. The patient was discharged on postoperative day 7 without adverse events.

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Total Arch Replacement for Acute Aortic Dissection (Stanford A) in a Patient With Hemophilia A

Cited by 7 publications

References 6 publications

Perioperative Coagulation Management of a Hemophilia A Patient During Cardiac Surgery

Perioperative Coagulation Management of a Hemophilia A Patient During Cardiac Surgery

Consensus Review of the Treatment of Cardiovascular Disease in People With Hemophilia A and B

Bentall Operation in a Patient With Severe Hemophilia A and Marfan Syndrome by Use of a Biologic Composite Graft

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