Assessment of von Willebrand disease and pregnancy outcomes at regional Australian hospitals

Abstract

Background von Willebrand disease (vWD) is a heterogeneous hereditary bleeding disorder and is associated with risk of primary postpartum haemorrhage (PPH). Design and Methods An observational study at a tertiary referral centre in Australia of 16 women with 23 deliveries with a median age of 27.5 years (range, 21‐39; IQR = 9). Median gestational age at delivery was 39 weeks (range, 35‐41; IQR = 1.1). Results All cases had type 1 vWD, apart from one case with type 2. Patients were managed in combined obstetrics and haematology clinics. PPH occurred in ten deliveries (44%). Intravenous desmopressin was administered in 6 cases, and IV human vWF was administered in 4 cases. Two cases with mild vWD had received oral tranexamic acid. The median Apgar score at 1 and 5 min was 9 (IQR = 1.0), while the median Apgar score at 10 min was 10.0 (IQR = 0.0). One case required transfusion of blood products postdelivery. There were no other significant complications observed. Conclusions vWD was associated with a high incidence of primary PPH. Individualised treatment to restore haemostasis, according to the severity of the disease, could achieve as possible, normal haemostasis with favourable outcomes for both mothers and their infants. Further studies to confirm our findings are warranted.

The Effect of Von Willebrand Disease on Pregnancy, Delivery, and Postpartum Period: A Retrospective Observational Study

“…The median APGAR score at 5 min was equal for infants born to women with VWD (9; IQR 9-9) and women without VWD (9; IQR 9-10). This finding is in agreement with an observational study by Wilson et al from Australia, who only included women with VWD (23 deliveries overall) and reported a median APGAR score at 5 min of nine [31].…”

Von Willebrand Disease Epidemiology, Burden of Illness and Management: A Systematic Review

“…15,33 The systematic review also highlighted high disease burden, with a high proportion of patients with VWD reporting a history of bleeding events. 23,28,31,34,37,39,[42][43][44][45]47,[54][55][56][57][58][59][60][61][62][63]67,68,[70][71][72][73] Patients with VWD were also reported to have lower/poorer HRQoL scores versus general populations, 77,80,82 as well as increased HCRU. 83,84 VWD prevalence estimates varied highly according to the study populations and method of estimation.…”

“…The studies reviewed suggest that HMB occurs frequently (>60%) in patients with VWD, 23,31,34,42,44,[54][55][56][57][58] while postpartum bleeding can occur in up to 13% of women with VWD. 42,47,50,[59][60][61][62][63] This systematic review also suggests that there are important gaps in VWD diagnosis that could delay care and result in adverse health outcomes. Although the time from symptom development to diagnosis was poorly documented in the literature, 38,52 the 3-year difference between median age at overt symptom onset and median age at diagnosis reported in this review 38 highlights the need for improved disease awareness for both patients and health care providers.…”

“…47,59 Postpartum hemorrhage occurred less frequently than HMB, occurring in 5.5-12.7% of the deliveries in women with VWD. 42,47,50,[59][60][61][62][63] Miscarriages were reported in 3-29% of the pregnant women with VWD. [64][65][66] Rates of anemia and iron deficiency in women with VWD (including pregnant women) ranged from 13% to 21.4% 50,52,65 and 4.2 to 22.5%.…”

“…On-demand treatments included desmopressin, replacement therapy with VWF (including VWF/FVIII concentrates), antifibrinolytics, and blood transfusion. 38,51,63,72,85,[87][88][89][90][91] There were 17 sources reporting on the use of situational/conditional prophylaxis (ie, for surgical procedures or childbirth), and nine sources on the use of long-term prophylaxis. Treatments for situational/ conditional prophylaxis included desmopressin, plasma-derived VWF and VWF/FVIII-containing concentrates (as monotherapy or in combination), recombinant VWF, and tranexamic acid.…”