Cardiac Surgery with Extracorporeal Circulation in Severe Factor VII Deficiency

Ferster, Alina; Capouet, Vincent; Deville, Andrée; Fondu, Pierre; Corazza, Francis

doi:10.1159/000216855

Cited by 15 publications

(11 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Recent work using the thrombin generation test (TGT) has suggested that levels of 2 U dL )1 are sufficient to normalize the TGT although this has not been used to monitor replacement therapy [105]. Factor VII concentrate -For surgery, doses of FVII concentrate ranging from 8 to 40 U kg )1 every 4-6 h have been successfully used [89,[106][107][108][109]. Careful monitoring of patients receiving FVII concentrates is essential to prevent excessively high peaks and troughs.…”

Section: Management Of Surgerymentioning

confidence: 99%

The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation

et al. 2004

View full text Add to dashboard Cite

Summary.The rare coagulation disorders are heritable abnormalities of haemostasis that may present significant difficulties in diagnosis and management. This review summarizes the current literature for disorders of fibrinogen, and deficiencies of prothrombin, factor V, FV + VIII, FVII, FX, the combined vitamin K-dependent factors, FXI and FXIII. Based on both collective clinical experience and the literature, guidelines for management of bleeding complications are suggested with specific advice for surgery, spontaneous bleeding, management of pregnancy and the neonate. We have chosen to include a section on Ehlers-Danlos Syndrome because haematologists may be consulted about bleeding manifestations in such patients.

show abstract

Section: Management Of Surgerymentioning

confidence: 99%

The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation

et al. 2004

View full text Add to dashboard Cite

show abstract

“…[5] However, a review of the literature revealed only four reported cases of open heart surgery in patients with FVII deficiency. [5] In all of these cases, the FVII deficiency was less than 10%, and the replacement therapy was carried out to maintain the plasma FVII concentration at approximately 15% to 25%. The administration varied from a single dose to multiple doses or continuous infusion from as low as 11-25 mcg/kg to as high as a cumulative dose of >400 mcg/kg.…”

Section: Discussionmentioning

confidence: 99%

Aortic valve replacement in a patient with factor VII deficiency

Uğuz¹

2012

Turk Gogus Kalp Dama

View full text Add to dashboard Cite

A 69-year-old man who had never experienced episodes of bleeding was admitted to our hospital with the complaints of dyspnea, palpitation and orthopnea. Electrocardiography, echocardiography and laboratory tests revealed atrial fibrillation, severe aortic stenosis and elevated INR level (2.18). Coagulation tests due to isolated INR elevation showed that the activity of factor VII (FVII) decreased to 9% and the patient was diagnosed with isolated FVII deficiency. As FVII activity<10% is associated with high risk of bleeding during surgical procedures, recombinant FVII (rFVIIa) 40 mcg/kg was administered intravenous bolus infusion two hours before surgery. Aortic valve replacement was performed using a-23 mm bioprosthesis. The activity level of FVII during anesthesia induction and aortic cross declamping were 29% and 22%, respectively. Hemostasis was easy to secure and no extra dose of rFVIIa was given. The patient did not experience any postoperative problem or severe bleeding. Warfarin sodium was not administered to the patient, as he had atrial fibrillation, as well as bioprosthesis implantation and an INR level of 2.5. At the end of a-two-year follow-up period, the patient had a good exercise tolerance without any bleeding or thromboembolic complication. Factor VII deficiency is an extremely rare inherited bleeding disorder. Replacement therapy with rFVIIa with close monitoring of FVII activity is a reliable way to manage patients with FVII deficiency who are scheduled to undergo valvular cardiac surgery.

show abstract

“…PCCs consist of other coagulation factors like II, IX, X and VIII, with which continuous infusion can have severe thrombogenic effects on patients who require continuous infusions. Effective management of FVIId patients has been proved by using virus inactivated FVII concentrates derived from plasma, even though theoretically it has a risk of infections transmitted due to transfusion [6,7].…”

Section: Introductionmentioning

confidence: 99%

Management of Arthropathy in Moderately Deficient Factor VII Patients: Results From Hospital Developed Treatment Protocol

Babu¹,

Belle²,

Pathiraj³

2016

J Hematol

View full text Add to dashboard Cite

Background: Factor VII deficiency (FVIId) is a rare inherited bleeding disorder whose prevalence is estimated to be 1 in 300,000 -500,000 in general population. In India, the epidemiological data on factor VII (FVII) disorder are scarce. Serious arthropathy is observed in cases which are severely affected with spontaneous bleeding and hemarthrosis. In such cases, orthopedic surgery is required. Fresh frozen plasma, prothrombin complex concentrates, factor VII concentrates -plasma derived and recombinant factor VII (rFVII) are different types of preparations which are available as therapeutic options in treatment for bleeding occurring during or after surgery in FVIId patients. The main objective of our study was to give a result of the treatment protocol for FVIId patients undergoing orthopedic surgery in our hospital and compare its results with other studies conducted around the world.

show abstract

Cardiac Surgery with Extracorporeal Circulation in Severe Factor VII Deficiency

Cited by 15 publications

References 0 publications

The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation

The rare coagulation disorders – review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation

Aortic valve replacement in a patient with factor VII deficiency

Management of Arthropathy in Moderately Deficient Factor VII Patients: Results From Hospital Developed Treatment Protocol

Contact Info

Product

Resources

About