Acute Type-A Dissection in a Patient with Severe Hemophilia A

Diplaris, Konstantinos; Karfis, Elias; Ampatzidou, Fotini; Ananiadou, Olga; Vakalopoulou, Sofia; Madesis, Athanasios; Asteri, T; Drossos, George

doi:10.1053/j.jvca.2011.07.017

Cited by 8 publications

(10 citation statements)

References 12 publications

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“… 58 , 125 In individuals with refractory bleeding during or after operation, acquired hemostatic perturbations, such as consumptive or dilutional coagulopathies from cardiopulmonary bypass (CPB) or deep hypothermia, are possibilities. 127 Cases complicated by acquired coagulopathy may require additional hemostatic products (eg, FFP, platelets) independent of hemophilia factor replacement.…”

Section: Cardiovascular Conditions Requiring Operations In People Witmentioning

confidence: 99%

“…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%

See 1 more Smart Citation

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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Section: Cardiovascular Conditions Requiring Operations In People Witmentioning

confidence: 99%

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

View full text Add to dashboard Cite

show abstract

“…Although several cases of cardiac operations in patients with mild or moderate forms of hemophilia A have been reported [3], few have undertaken cardiac or aortic operations in those with severe hemophilia A. Our patient had no factor inhibitor activity; thus, his requirements for factor VIII replacement were great.…”

Section: Commentmentioning

confidence: 72%

“…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: 74%

“…Few articles in the literature describe elective cardiac operations in hemophilia A patients. 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.…”

Section: Commentmentioning

confidence: 99%

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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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Internal carotid artery dissection in a patient with hemophilia A: a case report and literature review

et al. 2023

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Acute Type-A Dissection in a Patient with Severe Hemophilia A

Cited by 8 publications

References 12 publications

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

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

Internal carotid artery dissection in a patient with hemophilia A: a case report and literature review

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