In a congenital factor vii(fvii) deficient patient amniocentesis with recombinant fvii(rhfvii) was easily performed. surveillance by prothrombin time is very satisfactory

We report on a pregnant patient with a deficiency of 10-15%, nearly 38 years old. requesting at 4 months of pregnancy an epidural amniocentesis and if possible no blood products.The patient had not had any hemostatic problems before. She had given birth to a normal boy six years before under coverage of fresh frozen plasma.We strongly advised against an epidural procedure because of the unacceptable though rare risk of paraplegia.The availability of rhFVII (NovoSeven) made it possible to avoid blood products.Advantages and risks were discussed with the patient and her obstetrician. The manufacturer (NovoNordisk,Copenhagen- Denmark) advises in bleeding disorders an initial dose of 3-6KUI per kg in an i-v bolus, follow-up administrations to be judged by the treating physician.As amniotic fluid is thrombophilic.we gave about 15% of the calculated dose i.e. 30KUI total to the 54kg patient.FVII went from 21% to 430 %,PT/INR from 38%/l,9 to 100%/0,9 Amniocentesis was performed 30 minutes thereafter.After 5h FVII was 99% and PT/INR 87%/1, 1 ,the same dose was given.8h thereafter a same third dose was given when PT/INR were 76%/l ,2. Ten hours after the last dose PT/INR were 71 %/l ,2. FVII was 22% and PT/INR were 34%/2 36h after the last dose.Because of a thrombophilic situation during amniocentesis,the impossibility to calculate distribution volumes,we advise low doses of FVII.Blood levels are very high and clinically uninterpretable, PT/INR are relevant and easy titrable.