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von Willebrand disease (VWD): Gynecologic and obstetric considerations

von Willebrand disease (VWD): Gynecologic and obstetric considerations
Literature review current through: Jan 2024.
This topic last updated: Dec 14, 2023.

INTRODUCTION — von Willebrand disease (VWD) is the most common inherited bleeding disorder. Management can vary widely depending on the type of VWD, severity and location of bleeding, and need for invasive procedures.

This topic reviews the gynecologic and obstetric management of VWD.

Separate topic reviews discuss the treatment of bleeding and surgery, clinical presentation, diagnosis, and pathophysiology, and acquired von Willebrand syndrome (AVWS):

Treatment of major bleeding and surgery – (See "von Willebrand disease (VWD): Treatment of major bleeding and major surgery".)

Minor bleeding, DDAVP, and routine care – (See "von Willebrand disease (VWD): Treatment of minor bleeding, use of DDAVP, and routine preventive care".)

Diagnosis and classification of types – (See "Clinical presentation and diagnosis of von Willebrand disease".)

Pathophysiology – (See "Pathophysiology of von Willebrand disease".)

Diagnosis and treatment of AVWS – (See "Acquired von Willebrand syndrome".)

EFFECTS OF REPRODUCTIVE HORMONES ON VWF LEVELS — High levels of estrogen (such as occur during pregnancy) are associated with an increase in von Willebrand factor (VWF) and factor VIII (FVIII) levels; as a result, VWF and FVIII levels are higher during pregnancy and decline rapidly after delivery [1]. The impact of lactation on VWF levels has not been well studied. (See "Pathophysiology of von Willebrand disease", section on 'Synthesis and multimerization' and 'Delivery and postpartum care' below.)

The known effect of estrogen on VWF levels has led to questions about whether VWD testing should occur at a certain time during the menstrual cycle and whether estrogen-containing contraceptives may impact VWF levels.

A review of available studies found conflicting results regarding changes in VWF levels during the menstrual cycle [2]. It concluded that there was insufficient evidence to perform VWD testing at a specific phase of the menstrual cycle; however, if levels were borderline and the patient experienced heavy menstrual bleeding, it may be useful to repeat testing at day 3 of menses.

The review also stated that it is unlikely that the amount of estrogen in most oral contraceptives is sufficient to substantially increase VWF levels [2]. Thus, testing for VWD can reasonably be performed in an individual taking an estrogen-containing contraceptive. While there may be benefits of hormonal contraception in reducing menstrual blood loss and controlling menstrual pain, hormonal contraceptives would not be expected to raise VWF levels with most formulations. It is possible that individuals who require hormonal contraceptives with higher estrogen content to control heavy menstrual bleeding (HMB) may have a slight increase in VWF levels. (See 'Interventions for HMB' below.)

HEAVY MENSTRUAL BLEEDING

Assessing severity and other causes — Heavy menstrual bleeding (HMB) is common in VWD. (See "Clinical presentation and diagnosis of von Willebrand disease", section on 'Heavy menstrual bleeding'.)

A series of questions (table 1) can be helpful to assess the degree of blood loss before therapy is started. Interpretation of the replies and other pertinent questions are discussed in more detail separately. (See "Abnormal uterine bleeding in nonpregnant reproductive-age patients: Terminology, evaluation, and approach to diagnosis".)

Other causes of bleeding (eg, structural causes [uterine fibroids, endometrial polyps], anovulatory bleeding, endometrial carcinoma), should be considered and evaluated as appropriate. This is especially true when the pattern of bleeding differs from that individual's normal pattern. (See "Causes of female genital tract bleeding" and "Approach to the patient with postmenopausal uterine bleeding".)

Interventions for HMB — The following options can be discussed with the patient and an approach selected based on bleeding severity and patient preferences:

Hormonal contraception – Hormonal contraception (combined estrogen-progestin oral contraceptives or a levonorgestrel-releasing intrauterine device [LNG IUD]) may be used if pregnancy is not desired in the near future [3]. This may also be helpful for controlling painful menstruation, as nonsteroidal antiinflammatory drugs (NSAIDs) are generally avoided in VWD. (See "Hormonal contraception for menstrual suppression".)

The 2021 Guideline on management of VWD from the American Society of Hematology (ASH) suggests using combined hormonal contraception, a LNG IUD, or tranexamic acid to treat HMB [4]. For those who wish to conceive in the near future, and require therapy for HMB, tranexamic acid is preferred.

Antifibrinolytic agent or DDAVP – For those who prefer to avoid oral contraceptives due to desire to become pregnant in the near future or to avoid a daily pill, an antifibrinolytic agent or desmopressin (DDAVP) are options. In a crossover study of 116 females with heavy menstrual bleeding, tranexamic acid was more effective in lowering menstrual blood loss than DDAVP [5].

The antifibrinolytic agent should be used for the duration of menses. (See "von Willebrand disease (VWD): Treatment of minor bleeding, use of DDAVP, and routine preventive care", section on 'Antifibrinolytic agents'.)

Intranasal DDAVP can be used at the onset of menses to control heavy menstrual bleeding [5-7]. It is typically taken once daily on days 1 to 3 of menses. (See "von Willebrand disease (VWD): Treatment of minor bleeding, use of DDAVP, and routine preventive care", section on 'DDAVP'.)

Supporting evidence for these therapies comes from observational studies. Intranasal DDAVP appears to reduce blood loss from HMB, reduce need to seek medical attention, and reduce the number of days lost from work or school [5-7]. One prospective crossover study using tranexamic acid and intranasal DDAVP showed that both agents were effective, but tranexamic acid was superior to DDAVP in reducing bleeding in this setting [5].

VWF concentrates – If bleeding is more severe and/or is not adequately reduced with the measures listed above, von Willebrand factor (VWF) concentrate replacement therapy can be used at the onset of menstrual bleeding [8]. However, in a small randomized trial (36 patients) comparing recombinant VWF versus tranexamic acid for heavy menstrual bleeding, the recombinant VWF was not superior to tranexamic acid in reducing bleeding severity [9].

Ablation – Endometrial ablation is a minimally invasive procedure that can be used to surgically destroy the endometrium using various methods (eg, resection, radiofrequency, heat, cryotherapy). This is only appropriate for females who do not desire future childbearing. Several observational studies, including a 2009 meta-analysis of endometrial ablation in females with bleeding disorders, found the procedure to be safe and effective and comparable to an LNG IUD in reducing blood loss [10-12]. Indications, contraindications, and potential complications are discussed separately. (See "Overview of endometrial ablation".)

Hysterectomy – Hysterectomy is an option for patients who cannot be successfully managed with the strategies listed above, however, it is critical for a bleeding disorder expert to be involved in the perioperative management of the patient to minimize the risk of bleeding.

IRON DEFICIENCY

Screening for iron deficiency — Heavy menstrual bleeding and pregnancy can contribute to iron deficiency, which is common in females without bleeding disorders, especially following pregnancy.

Whom to screen

Menstruating or pregnant females with VWD should be screened for iron deficiency. (See "Anemia in pregnancy", section on 'Screening during pregnancy'.)

Other individuals may require screening based on other risk factors. (See "Iron deficiency in infants and children <12 years: Screening, prevention, clinical manifestations, and diagnosis", section on 'Screening recommendations' and "Iron requirements and iron deficiency in adolescents", section on 'Whom to screen'.)

Any individual with concern for iron deficiency should also be evaluated. (See "Causes and diagnosis of iron deficiency and iron deficiency anemia in adults", section on 'Causes and risk factors for iron deficiency'.)

How to screen – Typical screening is with a complete blood count (CBC) and ferritin level. Some clinicians may wish to obtain a CBC first and only order a ferritin level if there is anemia or microcytosis; however, early iron deficiency may not cause either of these findings (table 2). Some individuals with concomitant chronic inflammatory disorders may require a full iron studies panel or additional testing, as discussed separately. (See "Causes and diagnosis of iron deficiency and iron deficiency anemia in adults", section on 'Diagnostic evaluation'.)

Frequency of screening – We screen patients with heavy menstrual bleeding at least annually. Pregnant individuals are initially screened at their first prenatal visit and again in the third trimester. (See 'Prenatal testing' below.)

More frequent testing is reasonable if there are signs or symptoms of anemia or if severe bleeding occurs.

Treatment of iron deficiency — Management of iron deficiency involves repleting iron and addressing the underlying cause. (See "Iron deficiency in infants and children <12 years: Treatment" and "Iron requirements and iron deficiency in adolescents", section on 'Management' and "Treatment of iron deficiency anemia in adults".)

Iron products – Many iron formulations are available to treat iron deficiency. The choice of whether to use oral or intravenous iron depends on many factors, including available resources, tolerability, efficacy, and patient values and preferences, as summarized in the table (table 3).

Oral – Oral formulations are listed in the table (table 4).

Many individuals start with oral iron due to ease of use. Once every other day dosing (or taking one pill on Monday, Wednesday, and Friday) appears to provide similar or better efficacy than daily or multiple times per day dosing. (See "Treatment of iron deficiency anemia in adults", section on 'Oral iron'.)

Intravenous – Intravenous formulations are listed in the table (table 5).

Intravenous iron may be indicated if bleeding outpaces the ability to maintain iron stores with oral iron, if oral iron is not tolerated, during the second trimester of pregnancy if hemoglobin is <10.5 g/dL due to iron deficiency, or during the third trimester of pregnancy. For most of the products, the entire dose can be given in a single infusion. (See "Treatment of iron deficiency anemia in adults", section on 'Intravenous iron' and "Anemia in pregnancy", section on 'Treatment of iron deficiency'.)

Iron absorption – Dietary alterations generally are not required, and iron from foods is not sufficient to treat iron deficiency. Individuals who are adherent to oral iron therapy and appear not to be absorbing the iron may benefit from a review of their diet to ensure their dietary practices are not inadvertently interfering with absorption. (See "Treatment of iron deficiency anemia in adults", section on 'Role of diet'.)

Another important consideration is the possibility of a condition such as celiac disease or atrophic gastritis that is interfering with iron absorption and that would require additional therapies; if absorption is inadequate (hemoglobin or iron stores not improving), it is generally prudent to evaluate for these conditions. (See "Causes and diagnosis of iron deficiency and iron deficiency anemia in adults", section on 'Reduced iron absorption'.)

It is also possible that the initial diagnosis of iron deficiency was incorrect, or that another cause of anemia is also present. (See "Causes and diagnosis of iron deficiency and iron deficiency anemia in adults", section on 'Differential diagnosis'.)

Reducing bleeding – Heavy menstrual bleeding is a common cause of iron deficiency, and interventions listed above may improve iron stores. (See 'Interventions for HMB' above.)

Other sources of bleeding (eg, chronic epistaxis, gastrointestinal bleeding) should be addressed. (See "von Willebrand disease (VWD): Treatment of major bleeding and major surgery", section on 'Chronic bleeding (epistaxis, GI)'.)

For older individuals, the possibility of another source of bleeding such as a colorectal tumor should also be evaluated. (See "Causes and diagnosis of iron deficiency and iron deficiency anemia in adults", section on 'Search for source of blood and iron loss'.)

OBSTETRIC CONSIDERATIONS

Reproductive counseling — Reproductive counseling (and possibly patient and/or partner testing) may be appropriate for any individual considering childbearing. It is useful to obtain a personal and family bleeding history from that individual to determine the risk of VWD and to educate the individual about the likelihood of having an affected child and the likely severity. History should be obtained from both partners (or from a sperm or egg donor). (See "Genetic counseling: Family history interpretation and risk assessment".)

VWD is typically an autosomal dominant condition (table 6). Affected individuals inherit a pathogenic variant in the VWF gene from one parent, and they have a 50 percent chance of transmitting the variant to their children. Issues of incomplete penetrance and variable expressivity complicate the inheritance of type 1 VWD, and a positive family history may not always be present.

A person who inherits a pathogenic variant in the VWF gene from both parents can have type 3 VWD (virtual absence of von Willebrand factor [VWF]). Type 3 has historically been considered autosomal recessive but it can also be codominant [13]. Individuals with type 3 VWD can be either homozygous or compound heterozygotes. The offspring of a homozygote or compound heterozygote for disease-causing VWF variants will receive one of the abnormal genes, likely manifesting a milder phenotype.

Prenatal testing — Individuals with type 3 VWD, or those who have had a previous child with type 3 VWD, may choose to have prenatal diagnosis using chorionic villus sampling or amniocentesis [14]. The method used for molecular testing depends on the specific gene variant in the family, and it is important that maternal DNA be distinguished from fetal DNA. (See "Chorionic villus sampling" and "Diagnostic amniocentesis".)

Unless there is a diagnosed bleeding disorder in the father (or a personal or family history of bleeding on the paternal side), individuals with any type of VWD other than type 3 do not require prenatal testing.

Pregnancy

Pre-pregnancy interventions — When possible, the obstetrician, primary clinician, and a hematologist with expertise in the management of VWD should coordinate planning by reviewing the individual's clinical history prior to the pregnancy or as early as possible after pregnancy is established.

The main goals are to document the patient's bleeding phenotype, determine the baseline VWF activity and factor VIII activity, and discuss a plan for monitoring VWF levels and treatment of bleeding or any procedures if needed.

Patients with VWD are at increased risk of iron deficiency, which can have serious consequences for both maternal and fetal health. Screening for iron deficiency should be conducted and treatment initiated if needed [15]. There are more options for correction prior to conception, since intravenous iron is not used during the first trimester. (See "Anemia in pregnancy", section on 'Oral versus IV iron'.)

Diagnostic testing and monitoring during pregnancy — Ideally, initial diagnostic testing for VWD is not done during pregnancy because VWF levels will be increased over the individual's baseline. Low VWF antigen or activity are diagnostic and may provide useful information, but borderline or normal levels may result in inaccurate exclusion of the diagnosis. (See 'Effects of reproductive hormones on VWF levels' above.)

In either case, it is appropriate to retest six weeks or more after delivery to confirm or exclude the diagnosis and establish the individual's baseline. Genetic testing is generally not needed for type 1 patients but may be helpful in individuals with type 2 or type 3 disease. (See "Clinical presentation and diagnosis of von Willebrand disease", section on 'Laboratory testing'.)

For individuals with a known diagnosis of VWD, VWF activity and factor VIII activity (and platelet count for type 2B VWD patients) are monitored throughout the pregnancy and delivery. A typical schedule is testing once during the first trimester (at the initiation of care) and once during the third trimester (for delivery planning). It may be reasonable to test again closer to delivery (approximately 36 weeks) for some patients.

If screening for iron deficiency and anemia was not performed before conception, it should be done at the first prenatal visit. (See "Anemia in pregnancy", section on 'Screening during pregnancy'.)

Invasive procedures and treatment of bleeding — Therapies that can be used in pregnancy include VWF concentrates and antifibrinolytic agents. Previously, DDAVP was avoided until just prior to delivery, based on a theoretical possibility that DDAVP could initiate uterine contractions [16,17]. However, more experience has indicated that DDAVP may be used during pregnancy for prophylaxis before invasive procedures [4,18,19]. (See 'Delivery and postpartum care' below.)

Procedures such as amniocentesis and chorionic villus sampling are generally safe if levels of VWF and factor VIII activity are maintained at >50 IU/dL [20].

Vaginal bleeding during pregnancy is almost always due to an abnormality of placentation (or other anatomical cause) rather than to VWD. Hemostatic treatments can reduce bleeding but will not stop bleeding due to a cause unrelated to VWD. Bleeding in other locations (eg, due to trauma or injury) may also occur.

For those who require treatment for uterine bleeding during pregnancy, an antifibrinolytic agent or VWF concentrate to raise the VWF activity to ≥50 IU/dL can be considered, depending on the severity and underlying cause of bleeding. However, bleeding should not be attributed solely to VWD, and other appropriate investigations and interventions may be required.

Patients at risk for preeclampsia — Low dose aspirin may be used in patients with VWD, especially if their VWF and factor VIII levels have normalized during pregnancy. Indications for low dose aspirin for preeclampsia prevention are discussed separately. (See "Preeclampsia: Prevention", section on 'Low-dose aspirin'.)

The fetal VWD status is unlikely to be known during pregnancy. There is no evidence of fetal harm from maternal low dose aspirin use, and fetal bleeding would not be expected, although the question of fetal outcomes following maternal aspirin use has not been studied systematically.

Planning for delivery — Anesthesia consultation should be obtained prior to the onset of labor to discuss options for regional anesthesia or analgesia.

A VWF activity level of ≥50 IU/dL is considered adequate for neuraxial anesthesia [21]. The 2021 VWD guidelines include a target range of 50 to 150 IU/dL for neuraxial anesthesia [4]. (See 'Delivery and postpartum care' below.)

Maternal outcomes — Pregnancy is generally well-tolerated, and the majority of individuals with mild VWD do not require additional treatment during the pregnancy.

Since VWF levels typically rise two- to threefold over baseline during the second and third trimesters, many patients with VWD reach normal levels of both VWF and factor VIII at term [22]. However, the increase may not be as large in individuals with more severe VWD, and thrombocytopenia may worsen in type 2B VWD [16,22,23]. (See "Clinical presentation and diagnosis of von Willebrand disease", section on 'Changes with aging and pregnancy'.)

In a large database review from 2007 that described outcomes of more than 4000 pregnancies and deliveries in individuals with VWD, there was no increase in adverse fetal outcomes compared with controls [24]. However, the risks of antepartum bleeding, postpartum bleeding, and transfusion were increased (odds ratios [ORs] 10.2, 1.5, and 4.7, respectively). There were five deaths (0.12 percent), which is 10-fold greater than expected based on the non-VWD population. Higher rates of postpartum bleeding have been reported in many series, such as those discussed below. (See 'Delivery and postpartum care' below.)

Delivery and postpartum care — For delivery, especially if VWF levels have not normalized in pregnancy, the birth should occur at a center where VWF and factor VIII levels can be monitored and VWF and factor VIII replacement products can be administered if necessary. Knowledge of the type of VWD, baseline activities of factor VIII and VWF, current and prior responses to DDAVP, and past bleeding episodes is useful for guiding peripartum therapy [25-27].

Anesthesia – Neuraxial anesthesia may be considered when VWF and factor VIII levels are maintained above 50 IU/dL (and are continued at that level for at least six hours after anesthesia). The 2021 guideline from ASH suggests a target VWF activity of 50 to 150 IU/dL [4].

Delivery – Vaginal birth is appropriate for most individuals; cesarean birth is reserved for obstetric indications [28]. Regardless of the mode of delivery, it is recommended that levels of VWF and factor VIII be maintained at ≥50 IU/dL during delivery and for at least three to five days after delivery [20,29-31]. We suggest VWF concentrates and factor VIII concentrates, respectively, if VWF activity and factor VIII activity remain <50 IU/dL [16,32].

Postpartum – The postpartum period requires extra vigilance. VWF levels start to decline very soon after delivery (hours) and return to nonpregnant values within a very short time (approximately one week or earlier) [1]. (See 'Effects of reproductive hormones on VWF levels' above.)

Many individuals with VWD who do not require treatment during pregnancy may have serious bleeding shortly after delivery and during the one to three weeks postpartum and require treatment [33].

In a retrospective series of 106 deliveries, there were 11 instances of postpartum hemorrhage, representing approximately 10 percent of the cohort [27]. Of these, eight had cesarean births. Three individuals had received DDAVP, and one received a VWF concentrate prior to delivery. Transfusion was required in 4 percent of deliveries, and hysterectomy was performed in one due to retained placenta.

The impact of lactation on VWF levels has not been well studied.

Hemostatic therapies – These therapies (typically one of the three options) can be used to reduce bleeding risk in some cases (mostly moderate-to-severe type 1 and types 2 and 3 VWD):

Antifibrinolytic therapy – For most individuals with VWD, we suggest giving tranexamic acid to reduce the risk of postpartum bleeding. The 2021 ASH VWD guidelines suggest giving tranexamic acid to individuals with type 1 (as well as types 2 and 3) VWD during the postpartum period over not giving it [4]. Tranexamic acid is safe during breastfeeding [34].

Administration can be oral or intravenous. The oral dose is 25 mg/kg three times per day for 10 to 14 days; longer duration of administration may be appropriate for ongoing bleeding [4]. An example intravenous dose is 1 gram over 10 to 20 minutes (weight-based dosing, 10 to 15 mg/kg). Dosing can be repeated three times a day (oral) or every eight hours (intravenous). (See "von Willebrand disease (VWD): Treatment of minor bleeding, use of DDAVP, and routine preventive care", section on 'Antifibrinolytic agents'.)

Several observational studies have shown tranexamic acid to be effective in decreasing postpartum blood loss in VWD patients [35-37]. In one study of 34 individuals with VWD, tranexamic acid, given at the onset of labor, had similar efficacy when given alone or in addition to other hemostatic medications [36].

VWF concentrates – VWF concentrates may be used if VWF levels are <50 IU/dL and/or cannot be raised with DDAVP (or if there has been a decision to avoid DDAVP, or if bleeding is severe or prolonged). Use of VWF concentrates may be needed for prolonged postpartum bleeding, which can last for up to six weeks or even three months. (See "von Willebrand disease (VWD): Treatment of minor bleeding, use of DDAVP, and routine preventive care", section on 'VWF concentrates and factor VIII for minor bleeding'.)

DDAVP – In patients who are known to have an adequate VWF response, a single dose of DDAVP can be given close to the time of delivery (often after the cord is clamped). Repeated doses should not be used, as patients must restrict sodium intake to prevent hyponatremia, which is not ideal during lactation, if planned.

If DDAVP is prescribed, fluids should be restricted to maintenance levels only for 24 hours and serum sodium levels measured to avoid hyponatremia. (See "von Willebrand disease (VWD): Treatment of minor bleeding, use of DDAVP, and routine preventive care", section on 'DDAVP'.)

Avoid NSAIDs – Nonsteroidal antiinflammatory drugs (NSAIDs) should generally be avoided to reduce the possible contribution to increased bleeding risk.

Newborn — Newborns at risk of mild VWD should receive normal newborn care. Those at risk of more severe types of VWD should be seen by a pediatric hematologist.

Data regarding bleeding risk in newborns are extremely limited. Neonatal VWF and factor VIII activity levels tend to be higher than baseline for approximately six months; testing for mild VWD should be delayed until (or repeated after) six months of age [38].

Elective surgical procedures on the newborn such as circumcision should be delayed until the infant's VWF and factor VIII activity levels have been determined. (See "Neonatal circumcision: Risks and benefits".)

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: von Willebrand disease".)

PATIENT PERSPECTIVE TOPIC — Patient perspectives are provided for selected disorders to help clinicians better understand the patient experience and patient concerns. These narratives may offer insights into patient values and preferences not included in other UpToDate topics. (See "Patient perspective: von Willebrand disease".)

SUMMARY AND RECOMMENDATIONS

Hormonal effects – Estrogen is associated with increased von Willebrand factor (VWF) and factor VIII (FVIII) levels. (See 'Effects of reproductive hormones on VWF levels' above.)

Heavy menstrual bleeding – Heavy menstrual bleeding (HMB) is common. Questions can assess the degree of blood loss (table 1). Other causes of bleeding (eg, structural causes [uterine fibroids, endometrial polyps], anovulatory bleeding, endometrial carcinoma), should be considered and evaluated as appropriate, especially when the bleeding pattern differs from that individual's normal pattern. (See 'Heavy menstrual bleeding' above.)

For individuals with HMB who wish to become pregnant in the near future, we suggest tranexamic acid rather than desmopressin (DDAVP) (Grade 2C).

For individuals who do not wish to become pregnant in the near future, we suggest either combined hormonal contraception, a levonorgestrel-releasing intrauterine device (LNG IUD), or tranexamic acid rather than DDAVP (Grade 2C).

Severe bleeding can be treated with a VWF concentrate. Endometrial ablation and hysterectomy are options if fertility is not desired.

Iron deficiency – Iron deficiency is common in reproductive-age females. Menstruating females with VWD should be screened for iron deficiency with complete blood count (CBC) and ferritin at least annually; pregnant individuals are initially screened at their first prenatal visit and again in the third trimester. Many iron formulations are available for treatment, including oral (table 4) and intravenous iron (table 5). The table summarizes considerations for choice of formulation (table 3). Other evaluations may be indicated. (See 'Iron deficiency' above.)

Reproductive counseling and testing – VWD is usually autosomal dominant; type 3 (absent VWF) is autosomal recessive. Reproductive counseling is appropriate, but prenatal testing is generally restricted to individuals with a prior child with type 3 VWD or a diagnosed or suspected bleeding disorder in the father. (See 'Reproductive counseling' above and 'Prenatal testing' above.)

Pregnancy

Evaluation and planning – Pregnancy is generally well-tolerated. The obstetrician, primary clinician, and hematologist with expertise in VWD should coordinate planning. Ideally, initial diagnostic testing is not done during pregnancy. (See 'Pre-pregnancy interventions' above and 'Diagnostic testing and monitoring during pregnancy' above.)

Bleeding and procedures – Procedures such as amniocentesis and chorionic villus sampling are generally safe if VWF and FVIII activity are >50 IU/dL. Vaginal bleeding during pregnancy is almost always due to an abnormality of placentation (or other anatomical cause). (See 'Invasive procedures and treatment of bleeding' above and 'Planning for delivery' above and 'Maternal outcomes' above.)

Delivery – Vaginal birth is usually appropriate. VWF activity ≥50 IU/dL is generally considered adequate for neuraxial anesthesia. Delivery should occur at a center where VWF and FVIII levels can be monitored and replacement products administered if needed, since VWF levels can decrease within hours after delivery. (See 'Delivery and postpartum care' above.)

-For most individuals, we suggest tranexamic acid (Grade 2C). Administration can be oral or intravenous (typical oral dose, 25 mg/kg three times daily for 10 to 14 days; example intravenous dose, 1 gram over 10 to 20 minutes [weight-based dose 10 to 15 mg/kg] every eight hours).

-Alternatives include reserving therapy for bleeding or administering a single dose of DDAVP (if a prior response has been demonstrated). DDAVP is often given after the cord is clamped, and fluids must be restricted. VWF concentrates may be needed, in some cases for a prolonged duration.

Newborn – Newborns at risk of mild VWD should receive usual care; those at risk for severe disease should be seen by a pediatric hematologist. Testing for VWD should be delayed until (or repeated after) six months of age for an accurate baseline. Procedures such as circumcision should be delayed until VWF and FVIII activity have been determined. (See 'Newborn' above.)

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Topic 139017 Version 8.0

References

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