Hemophilia A and C in a female: The first case report in literature

Alkarrash, Mohamad Shadi; Badawi, Rayan; Sallah, Hala; Shashaa, Mohammad Nour; Argilo, Jerair; Alkhoury, Rawad

doi:10.1016/j.amsu.2021.102561

Cited by 1 publication

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“…Combined FVIII and FXI deficiency is an extremely rare disorder and is called the first group of Familial Multiple Coagulation Factor Deficiencies, which was only reported in 10 male and one female patient and were not related to a surgery. 8 , 9 Although the bleeding tendency of FXI deficiency is generally mild, severe intractable bleeding may be provoked by the combined effect of FVIII deficiency perioperatively. The present coagulation management for off-pump CABG based on the protocol achieved perioperative target levels of the FVIII:C and FXI:C values.…”

Section: Discussionmentioning

confidence: 99%

Hemostatic protocol and risk-reduction surgery for treating coronary artery disease with aortic stenosis in a patient with combined coagulation factor VIII and XI deficiency: a case report

Honda

Shimahara

Chikasawa

et al. 2023

European Heart Journal - Case Reports

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Background Cardiac surgery remains a significant challenge in patients with coagulation factor VIII (FVIII) deficiency, especially, in those with multiple factor deficiencies. Case summary A 79-year-old man with combined FVIII and factor FXI (FXI) deficiency was admitted for heart failure treatment. Transthoracic echocardiography revealed aortic stenosis with decreased left ventricular ejection fraction (LVEF) of 40%, mean aortic pressure gradient of 21 mmHg, and aortic valve area of 0.58 cm2. Coronary angiography revealed significant triple-vessel disease. The patient had multiple comorbidities, including diabetic end-stage renal disease treated with hemodialysis and liver cirrhosis (Child–Pugh score of A). Considering the high surgical risk, a two-stage treatment strategy was developed: the first with off-pump coronary artery bypass grafting (CABG), and the second with transcatheter aortic valve implantation if aortic stenosis symptoms were significant after CABG. A perioperative hemostatic protocol by the author’s heart team was used to appropriately replenish recombinant FVIII concentrates and fresh frozen plasma. The target preoperative and postoperative FVIII coagulation activity values were set at 80%–100% and 60%-80%, respectively, whereas the target perioperative FXI coagulation activity value was set at 30%–45%. Off-pump CABG without aortic manipulation was completed without bleeding events. Transthoracic echocardiography conducted 20 months postoperatively revealed LVEF of 65% and mean aortic pressure gradient of 31 mmHg. The patient leads a normal life 21 months after surgery. Discussion The hemostatic protocol and risk-reduction surgery provided satisfactory surgical results in a patient with significant coronary artery disease and aortic stenosis, high-surgical-risks, and combined FVIII and FXI deficiency.

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Section: Discussionmentioning

confidence: 99%