Updated Australian consensus statement on management of inherited bleeding disorders in pregnancy

Abstract

IntroductionThere have been significant advances in the understanding of the management of inherited bleeding disorders in pregnancy since the last Australian Haemophilia Centre Directors’ Organisation (AHCDO) consensus statement was published in 2009. This updated consensus statement provides practical information for clinicians managing pregnant women who have, or carry a gene for, inherited bleeding disorders, and their potentially affected infants. It represents the consensus opinion of all AHCDO members; where evidence was lacking, recommendations have been based on clinical experience and consensus opinion.Main recommendationsDuring pregnancy and delivery, women with inherited bleeding disorders may be exposed to haemostatic challenges. Women with inherited bleeding disorders, and their potentially affected infants, need specialised care during pregnancy, delivery, and postpartum, and should be managed by a multidisciplinary team that includes at a minimum an obstetrician, anaesthetist, paediatrician or neonatologist, and haematologist. Recommendations on management of pregnancy, labour, delivery, obstetric anaesthesia and postpartum care, including reducing and treating postpartum haemorrhage, are included. The management of infants known to have or be at risk of an inherited bleeding disorder is also covered.Changes in management as a result of this statementKey changes in this update include the addition of a summary of the expected physiological changes in coagulation factors and phenotypic severity of bleeding disorders in pregnancy; a flow chart for the recommended clinical management during pregnancy and delivery; guidance for the use of regional anaesthetic; and prophylactic treatment recommendations including concomitant tranexamic acid.

How I manage pregnancy in carriers of hemophilia and patients with von Willebrand disease

“…Instrumental vaginal deliveries should be avoided as much as possible, but if unavoidable, forceps delivery is preferred over ventouse delivery. 17 As mentioned earlier, we recommend an unassisted vaginal delivery with an early recourse to an emergency cesarean delivery if the second stage of labor is prolonged or there is suspected fetal distress. 17,18 The use of potentially harmful invasive techniques, like fetal scalp electrodes or fetal blood sampling, is avoided, as well as intramuscular injections in the neonate after birth: this is so-called atraumatic management ( Figure 1).…”

“…If, after preconception counseling, a couple decides they want to avoid the risk of having an affected child, we inform them about the possibilities of preimplantation genetic screening or antenatal genetic screening by chorionic villus sampling (weeks 10-14 of gestation) or amniocentesis (from week 15 of gestation) followed by (early) termination of pregnancy in case of an affected male fetus. [16][17][18] The latter is only possible if the causative mutation is known and if the national laws governing such procedures make this possible.…”

“…Because most female carrier newborns will have normal or only mild reduced FVIII/FIX levels and severe reduced factor levels are very rare, delivery of a (possible) carrier of hemophilia does not carry a risk of neonatal bleeding. 17,18 A male fetus has a 50% chance of being affected with hemophilia. When the causative mutation is known, it is possible to determine antenatally whether the boy is affected or not by analyzing fetal DNA obtained by chorionic villus sampling or amniocentesis.…”

“…41,42 In many guidelines on hemophilia carriers, factor levels in the third trimester $50 IU/dL are considered sufficient for delivery; below 50 IU/dL, administration of factor concentrates is warranted during delivery. 17,18,28,29 As an alternative to FVIII concentrate, desmopressin (DDAVP) can be administered in carriers of HA. Desmopressin can be safely administered in the first and second trimesters of pregnancy, however, its safety has not been demonstrated at the time of delivery.…”

“…We maintain FVIII/FIX and VWF levels .50 IU/dL for the duration of catheter placement and for at least 12 hours (mild hemophilia) to 24 hours (moderate or severe hemophilia) after catheter removal. 17,28,74 However, in the recent UK guideline, neuraxial anesthesia is not recommended for use in women with type 2 or 3 VWD irrespective of whether VWF activity has been restored to apparently normal levels. 59 In carriers of hemophilia or women with VWD undergoing cesarean delivery receiving FVIII (/VWF) concentrate or desmopressin, we consider thrombosis prophylaxis with low-molecular-weight heparin during hospital stay or longer if there is prolonged immobilization, following the Dutch guidelines (https://richtlijnendatabase.nl/ richtlijn/antitrombotisch_beleid).…”