Primary postpartum hemorrhage (PPH) remains a significant complication for pregnant women with type 3 von Willebrand disease (VWD), according to results from a systematic literature review published in the American Journal of Perinatology.

VWD is a hereditary bleeding disorder, and type 3 is both the most severe and the rarest. The authors of this literature review aimed to review maternal characteristics, challenges for management, and complications in pregnant women with type 3 VWD.

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Researchers conducted a systematic literature review via PubMed, Scopus, and Cochrane Central Register of Controlled Trials of articles published between January 1982 and May 2019. Identified articles were published in English and pertained to VWD and pregnancy without limitations on time or study.

A total of 13 studies met the inclusion criteria, representing 28 pregnancies in 17 women with type 3 VWD. All patients were diagnosed with type 3 VWD before pregnancy.

Treatment for VWD involves replacement of missing or malfunctioning clotting factors, with predelivery treatment recommended for pregnant women with VWD when hemorrhage is anticipated.

In 19 pregnancies, patients received concentrate treatment prior to delivery. In 26 pregnancies, patients received concentrate treatment after delivery. In 8 pregnancies, blood products were required after delivery.

PPH occurred in nearly half of pregnancies for which it was reported (48%; 10/21). Secondary PPH occurred in more than half of pregnancies for which it was reported (56%; 5/9) and occurred between 7 and 22 days after delivery.

The researchers suggested that the women who experienced PPH had a history of bleeding manifestations. However, they also noted that their review highlighted “the missing standardized approach regarding timing or type of treatment among women with type 3 VWD during pregnancy.”

None of the studies reported hysterectomies, admissions to the intensive care unit, or maternal mortality.

All 28 pregnancies resulted in 28 at-term live births. All pregnancies were singleton pregnancies. Cesarean delivery occurred in 57% (16/28) of pregnancies. Indications for cesarean delivery included elective (5 pregnancies), malpresentation (3 pregnancies), arrest of descent (3 pregnancies), failed labor induction (2 pregnancies), and severe bleeding (1 pregnancy).

Reference

  1. Makhamreh MM, Kass SL, Russo ML, Ahmadzia H, Al-Kouatly HB. Type 3 von Willebrand disease in pregnancy: a systematic literature review [published online November 22, 2019]. Am J Perinatol. doi:10.1055/s-0039-1700541