Pregnancy outcomes in women with vWD are generally good, but postpartum hemorrhage is encountered in up to half of cases. In a fourth of 38 cases summarized by Conti and associates (1986), bleeding was reported with abortion, with delivery, or in the puerperium. Kadir and coworkers (1998) reported their experiences with 84 pregnancies. They described a 20-percent incidence of immediate postpartum hemorrhage and another 20-percent incidence of late hemorrhage. Most cases were associated with low vWF levels in untreated women, and none given treatment peripartum had hemorrhage. More recently, Stoof and colleagues (2015) reviewed 185 deliveries in 154 afected women and found the risk for postpartum hemorrhage to be highest in deliveries with lowest factor levels.
Although most patients with vWD have heterozygous variants and associated minor bleeding complications, the disease can be severe. Moreover, homozygous ofspring develop serious clotting dysfunction. Chorionic villus sampling with DNA analysis to detect the missing genes has been described. Some authorities recommend cesarean delivery to avoid trauma to a possibly afected fetus if the mother has severe disease.
In general, the activity of most procoagulant factors rises across pregnancy (Appendix, p. 1256). Factor VII deiciency is a rare autosomal recessive disorder. Levels of this factor normally increase during pregnancy, but these may rise only mildly in women with factor VII deficiency (Fadel, 1989). A systematic review of 94 births found no diference in postpartum hemorrhage rates with or without prophylaxis with recombinant factor VIla (Baumann Kreuziger, 2013).
Factor X or Stuart-Prower actor deiciency is rare and is inherited as an autosomal recessive trait. Factor X levels typically rise by 50 percent during normal pregnancy. Konje and colleagues (1994) described a woman who had 2-percent factor activity. She was given prophylactic treatment with plasmaderived factor X, which raised her plasma levels to 37 percent. Despite this, she sufered an intrapartum placental abruption.
plasma to a woman with less than I-percent factor X activity.
She delivered spontaneously without incident. Beksa: and asso ciates (20r10) described a woman with severe factor X deiciency who was successfully managed with prophylactic prothrombin complex concentrate. Nance and colleagues (2012) reported on 24 pregnancies, of which 18 resulted in a healthy baby.
Factor I-plasma thromboplastin antecedent-diciency is inherited as an autosomal trait. It manifests as severe disease in homozygotes but only as a minor defect in heterozygotes. It is most prevalent in Ashkenazi Jews and is rarely seen in pregnancy. Musclow and coworkers (1987) reported 41 deliveries in 17 afected women, and none required transfusion. In 105 pregnancies from 33 afected women, Myers and colleagues (2007) reported an uneventful pregnancy and delivery in 70 percent. hey recommended peripartum treatment with factor XI concentrate if cesarean delivery is performed and advised against epidural analgesia unless factor XI is given. From their review, Martin-Sakes and associates (2010) found that factor XI levels and bleeding severity correlated poorly in women with severe deiciency. Wiewel-Verschueren and colleagues (2015) performed a systematic review of 27 studies with 372 women and reported that 18 percent had postpartum hemorrhage.
Factor II diciency is another autosomal recessive disorder that rarely complicates pregnancy. A greater incidence of thromboembolism is encountered in nonpregnant patients with this deficiency. Lao and coworkers (1991) reported an afected pregnant woman in whom placental abruption developed at 26 weeks' gestation.