Pontages coronariens avec circulation extracorporelle chez un patient atteint d'une hémophilie B

Bukowski, J.G.; Brux, JL De; Ganascia, B; Cottineau, C.; Jacob, J.P.

doi:10.1016/s0750-7658(96)80009-8

Cited by 6 publications

(2 citation statements)

References 7 publications

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“…Rossi and colleagues reviewed 84 papers regarding patients with HB and HA undergoing cardiac surgery [13]. They identified only 12 reports on HB patients, of those, only one [14] is about a cardiac surgery with EC in a patient with mild HB treated with rFIX concentrate. More recently, Curtin reported 21 patients with HB undergoing major or minor surgeries, demonstrating that EHL rFIX could be used during surgery, but no one underwent cardiac surgery in EC [15].…”

Section: Discussionmentioning

confidence: 99%

Successful Multidisciplinary Management of Aortic Valve Repair in Severe Hemophilia B with Extended Half-Life Recombinant Factor IX Concentrate

et al. 2023

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Successful management of surgery in severe coagulation disorders depends on adequate replacement of the deficient factors from intervention until wound healing. Extended half-life (EHL) recombinant factor IX (rFIX) has been increasingly used in hemophilia B (HB) patients. Monitoring of blood levels of EHL rFIX allows to obtain pharmacokinetic (PK) parameters in order to optimize and personalize therapeutic scheme. We describe a case of a young male with severe HB who successfully underwent aortic valve re-pair. This is the first reported open-heart surgery in a patient with severe HB using EHL rFIX. The success was based on accurate PK evaluation and on meticulous preoperative planning and close cooperation among surgeons, hemophilia specialists and laboratory team despite the long distance between hemo-philia center and surgical clinic.

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Section: Discussionmentioning

confidence: 99%

Successful Multidisciplinary Management of Aortic Valve Repair in Severe Hemophilia B with Extended Half-Life Recombinant Factor IX Concentrate

et al. 2023

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“…To our knowledge, this is the first case involving the management of haemophilia A patient using the off-pump technique and continuous FVIII concentrate infusion [1][2][3][4][5][6][7].…”

Section: Referencesmentioning

confidence: 98%

Off‐pump myocardial revascularization in a patient with haemophilia A: a case report and operative strategies

et al. 2011

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References1 Manco-Johnson MJ, Abshire TC, Shapiro AD et al. Recombinant factor VIII for the prevention of joint disease in children with severe hemophilia: prophylaxis compared with episodic treatment. N Engl J Med 2007; 357: 535-44. 2 Gringeri A, Lundin B, von Mackensen S et al. A randomized clinical trial of prophylaxis in children with hemophilia A (the ESPRIT Study). J Thromb Haemost 2011; 9: 700-10. 3 Mancuso ME, Graca L, Auerswald G et al. Haemophilia care in children -benefits of early prophylaxis for inhibitor prevention. Haemophilia 2009; 15(Suppl 1): 8-14. 4 Gouw S.C., van den Berg H.M. The multifactorial etiology of inhibitor development in hemophilia: genetics and environment. Semin Thromb Hemost 2009; 35: 723-34. 5 Pfistershammer K, Stö ckl J, Siekmann J et al. Recombinant FVIII and FVIII/VWF complex do not present danger signals for human dendritic cells. Thromb Haemost 2006; 96: 309-16. 6 Reipert BM, van Helden PM, Schwarz HP et al. Mechanisms of action of immune tolerance induction against factor VIII in patients with congenital haemophilia A and factor VIII inhibitors. Br J Haematol 2007; 136: 12-25. 7 Kurnik K., Bidlingmaier C, Engl W. et al. New early prophylaxis regimen that avoids immunological danger signals can reduce FVIII inhibitor development. Haemophilia 2010; 16: 256-62.Over the last 20 years the quality of life of haemophilia patients has improved considerably because of their management by specialist Haemophilia Centres (HC), improved safety of haemophilia drugs and the ability to offer patients prophylactic treatment. This increase in life expectancy is leading to an increased incidence of ischaemic heart disease and therefore of revascularization surgery. This is considered to be a 'major' surgery in severe haemophilia patients because of the perioperative risks of bleeding and thrombosis. We described the case of a patient who underwent dual off-pump aorto-coronary bypass grafting with continuous coagulation factor administration, and reviewed the literature on surgical strategies for aorto-coronary bypass procedures in haemophilia patients. A 38-year-old male patient suffering from severe haemophilia A [factor VIII (FVIII) < 1%], without anti-factor VIII inhibitor, was admitted to the cardiology intensive care unit with an ST elevation acute coronary syndrome, unsuitable for angioplasty (onset of symptoms 2 days previously). Dual aorto-coronary bypass grafting was recommended in view of his coronary angiography results. After discussion between cardiologists, the HC physician, anaesthetists and surgeon, we decided on off-pump surgery. A continuous FVIII Helixate NexGen Ò (Behring) infusion protocol was set up in conjunction with the HC. Thirty minutes before the surgical incision, the patient was given a bolus dose of 50 IU kg )1 of FVIII concentrate through a dedicated venous line (Fig. 1). FVIII levels at the end of the injection were 79% (Automate STAR, Diagnostica Stago, Asnières, France) Catheters were inserted immediately after induction, the arterial catheter into the radi...

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Myocardial Infarction, Other Arterial Thrombosis and Invasive Coronary Procedures, in Hemaophilia B: A Critical Evaluation of Reported Cases

Girolami

Randi

Ruzzon

et al. 2005

J Thromb Thrombolysis

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Myocardial infarction and other arterial thrombosis are commonly maintained to be rare in hemophilia patients. This, in general, seems true but the occurrence of a thrombotic event in hemophilia B is not exceptional. A thorough search of the literature has yielded 13 patients with myocardial infarction and 1 patient with a cerebrovascular accident. There were three fatalities. In five cases MI occurred after infusion of Prothrombin Complex Concentrates. In three additional patients the event occurred after infusion of plasma, Feiba or cryoprecipitate supernatant. Four patients had an antero-lateral infarction. Two had a non-Q infarction and one each showed a multiple or a posterior-inferior form. Several therapeutic coronary procedures (GABG and PTCA) were carried out in hemophilia B patients without undue complication providing adequate level of FIX were maintained. Heparin prophilaxis was used in all patients but one. The analysis of the literature indicates that (1) MI may occur in hemophilia B patients and (2) that invasive coronary artery therapeutic procedures may be carried out without complications.

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Pontages coronariens avec circulation extracorporelle chez un patient atteint d'une hémophilie B

Cited by 6 publications

References 7 publications

Successful Multidisciplinary Management of Aortic Valve Repair in Severe Hemophilia B with Extended Half-Life Recombinant Factor IX Concentrate

Successful Multidisciplinary Management of Aortic Valve Repair in Severe Hemophilia B with Extended Half-Life Recombinant Factor IX Concentrate

Off‐pump myocardial revascularization in a patient with haemophilia A: a case report and operative strategies

Myocardial Infarction, Other Arterial Thrombosis and Invasive Coronary Procedures, in Hemaophilia B: A Critical Evaluation of Reported Cases

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