OBSTETRICS

Evaluation and management of severe preeclampsia before 34 weeks' gestation

For many women, pregnancy and childbirth represent their first major hemostatic challenges. Despite advancements in obstetric care, up to 2 to 5% of all deliveries are complicated by postpartum hemorrhage (PPH). To mitigate bleeding risk, physiological changes occur in pregnancy, including increases in plasma von Willebrand factor (VWF) and factor VIII levels. For women with von Willebrand disease (VWD), these physiological alterations are blunted or absent. As a result, women with VWD have a heightened risk of PPH, both primary (in the first 24 hours) and secondary (>24 hours to 6 to 12 weeks postpartum). Pregnancy and delivery management for women with VWD should therefore be carefully coordinated as part of a multidisciplinary team approach. In the absence of large-scale clinical trials, the management of women with VWD during pregnancy is guided by expert consensus guidelines. Clinical practices internationally are not uniform, and areas of considerable clinical uncertainty exist. Traditional peripartum plasma VWF thresholds for hemostatic cover and therapeutic targets are currently under scrutiny, as PPH is not eliminated in women with VWD who receive replacement therapy. The benefit and optimal duration of postpartum tranexamic acid have yet to be defined, and standardized methods of quantification of blood loss at the time of delivery are currently lacking. In this article, we review the evidence base to date and explore the current clinical challenges in the management of pregnant women with VWD.

Examining international practices in the management of pregnant women with von Willebrand disease

Von Willebrand disease and other bleeding disorders in women: consensus on diagnosis and management from an international expert panel

Diagnosis and management of von Willebrand disease: A community-wide effort to deliver evidence-based clinical practice guidelines.

Outcome for Mothers and Neonates Among Pregnant Women with Von Willebrand Disease: A Prospective Nationwide Multicenter Study

Most maternal and newborn deaths in low-income countries, including Rwanda, are attributable to preventable causes. Timely access to Basic Emergency Obstetric and Newborn Care (BEmONC) guidelines to support clinical decisions could lead to better obstetric care thus reduction of maternal and newborn deaths. Besides, innovative methods such as the usage and reference to healthcare guidelines using mobile devices (mhealth) may support clinical decision making. However, there is little evidence about mhealth that focuses on the clinical decision support process. This proposal aims to investigate the effect of the Safe Delivery mhealth Application(SDA) on nurses’ and midwives’ clinical decision making, so as to inform mhealth interventions for work in specific contexts. The study adopts a quasi-experimental design. Convergent parallel mixed – methods will be used to collect, analyze and interpret data. A pre-intervention assessment of the BEmONC outcomes: Apgar score and PPH progressions, and related knowledge, skills, and perceptions of nurses and midwives will be conducted. The intervention will take place in two district hospitals in Rwanda and entails the implementation of the SDA for six months. Six months’ post-intervention, the effect of the SDA on BEmONC outcomes and the nurses’ and midwives’ knowledge and skills will be evaluated.

Postpartum Hemorrhage In Women With Von Willebrand Disease and Other Bleeding Disorders

Recognition and management of vascular lesions in von Willebrand disease

A literature review and best practice advice for second and third trimester risk stratification, monitoring, and management of pre‐eclampsia

To assess the obstetric outcome in women with von Willebrand's disease or factor XI deficiency. Haemophilia Centre and Haemostasis Unit, The Royal Free Hospital. Women with von Willebrand's disease (n = 31) and with factor XI deficiency (n = 11) registered at the Royal Free Hospital Haemophilia Centre who had had a pregnancy within the previous 17 years (1980-1996), including 84 in women with von Willebrand's disease and 28 in women with factor XI deficiency. Women were interviewed and details of the obstetric history were obtained. The records of the Haemophilia Centre and the women's maternity records were also reviewed. Threatened miscarriage occurred in 33% and 14% of pregnancies with von Willebrand's disease and factor XI deficiency, respectively. Excluding recurrent miscarriages, 14/68 (21%) of pregnancies with von Willebrand's disease and one pregnancy with factor XI deficiency miscarried spontaneously. There was an increased incidence of primary and secondary post-abortal bleeding complications. Factor VIII and von Willebrand factor antigen and activity levels increased significantly in pregnancy in all women apart from those with severe von Willebrand's disease. Factor XI, however, did not show any significant change. No neonatal haemorrhagic complications in association with the birth process were reported, although ventouse and difficult forceps deliveries were avoided. Extensive perineal bruising and haematoma was reported in three women with von Willebrand's disease; two of these were associated with forceps delivery. The incidence of primary postpartum haemorrhage was 18.5% in von Willebrand's disease and 16% in factor XI deficiency. Blood transfusion was required in six cases of von Willebrand's disease and two cases of factor XI deficiency. Ten of fourteen instances of primary postpartum haemorrhage occurred when maternal factor levels were < 50 IU/dL with no prophylactic treatment for labour. The incidence of secondary postpartum haemorrhage was 20% in von Willebrand's disease and 24% in factor XI deficiency. None of the women who had prophylactic treatment during labour or the puerperium suffered any significant bleeding complications. There were three neonatal bleeding complications. Pregnancy, labour and the puerperium are associated with significant bleeding problems in women with von Willebrand's disease or factor XI deficiency, but these are largely preventable. Specialist obstetric care in close liaison with the haemophilia centre is essential to minimise maternal and neonatal complications.

von Willebrand disease (VWD) is a hereditary bleeding disorder. Type 3 VWD is the most severe and rare phenotype that presents many challenges for management of pregnant women. The aim of this study was to review the maternal characteristics and complications in pregnant women with Type 3 VWD. A systematic literature search was performed to include all publications that address Type 3 VWD in pregnancy. Thirteen studies met the inclusion criteria. There were 28 pregnancies with Type 3 VWD in 17 women. All were diagnosed with Type 3 VWD prior to pregnancy. Concentrate treatment was administered before delivery for 19 pregnancies and postpartum for 26 pregnancies. Eight pregnancies required blood products postpartum. Primary postpartum hemorrhage (PPH) was reported in 48% (10/21) and secondary PPH was reported in 56% (5/9). Secondary PPH occurred between 7 and 22 days. No study reported hysterectomies, intensive care unit admissions, or maternal mortality. All 28 pregnancies resulted in 28 live births at term. Our review highlights the maternal outcomes in patients with Type 3 VWD and the different approaches in management during pregnancy and delivery. Despite prior knowledge of this bleeding disorder, PPH was still a significant complication.

To determine the current management of severe pre-eclampsia and eclampsia in the United Kingdom. One-page postal survey to all (1007) UK consultant obstetricians with questions about use of antihypertensive and anticonvulsant drugs in severe pre-eclampsia and eclampsia, other management strategies, definition of factors determining severity, protocol development and regional review. 688 replies (69.6% response rate). The antihypertensive drugs used were mainly oral labetalol (35%), oral methyl dopa (23%) and parenteral hydralazine (29%); diuretics were not used. Diazepam was the preferred drug in eclampsia. Very few consultants used magnesium sulphate (2%). Anticonvulsants were also prescribed by 85% of consultants to prevent fits; the drugs then preferred were diazepam (41%), phenytoin (30%) and chlormethiazole (24%). Two-thirds of consultants felt there was a need for trials to study the effectiveness of antihypertensive and anticonvulsant drugs. In a woman with proteinuric hypertension, 15% of consultants did not regard the development of headache as indicating severe pre-eclampsia. Consistent management practices were not associated with agreement about protocols. Regional review does not appear to have occurred. Antihypertensive and anticonvulsant therapies are widely used but trials are considered necessary. Improvements in the management of women with severe pre-eclampsia or eclampsia might occur if UK obstetricians sought more collective opinion and undertook regional audit of protocols.

Treatment of Von Willebrand Disease Women Undergoing Childbirth with a Von Willebrand Factor Product with a Low Content of Factor VIII: Results From 4 Multicenter Studies

While certain plasma-derived factor VIII/von Willebrand factor (FVIII/vWF) concentrates have proven to be quite useful in preventing or controlling bleeding in persons with von Willebrand disease who are not candidates for DDAVP, most of the information concerning dosage and effectiveness has been anecdotal. Additionally, the laboratory tests used to quantify vWF (the vWF:RCoF assay and collagen binding assay) are not well standardized. Thus, the US Food and Drug Administration (FDA) has been reluctant to grant licensed indication for use of these products in von Willebrand disease. This brief report describes a survey of non-US physicians (from major centres) who are recognized experts in haemophilia and von Willebrand disease. Twenty-four of 27 questionnaires were completed, returned and analysed. Products thought to be effective in von Willebrand disease included Haemate P, Facteur von Willebrand, Alphanate, and the UK's BPL '8Y'. In calculating dosage to stop or prevent bleeding, most aim for a certain level of both FVII and vWF:RCoF. Postoperatively, 16/24 respondents follow both of these laboratory tests once daily. Twenty-two of 24 would follow FVIII levels once daily. It is noteworthy that FVIII assay results are generally the only test results readily available to guide the respondents' clinical decisions. For treatment of significant mucous membrane bleeding, respondents often individualize dosage and monitoring, depending on the type, location and extent of bleeding. Gastrointestinal bleeding is generally treated more aggressively. Patient monitoring varies between merely looking for cessation of bleeding, to a battery of laboratory tests, including haemoglobin and haematocrit. Seven out of 24 would monitor BT as well. In addition to FVIII content, 22/24 respondents noted that they would find it helpful to have vWF:RCoF listed on the label, while 15/24 would like to know the vWF multimeric composition.