Von willebrand factor and pregnancy

Question 1

What is the inheritance of von willebrand disease?

Answer 1

Type 1 and 2 = dominant
Type 2N = recessive
Type 3 = recessive

Question 2

Which chromosome is von willebrand disease inherited from?

Answer 2

autosomal inheritance from chromosome 9

Question 3

What are the features of type 1 VWD?

Answer 3

Partial quantitative deficiency

e.g. <0.3iu/ml

Question 4

What are the features of type 2 VWD?

Answer 4

Variable presentation - qualitative deficiency in VWF activity

Question 5

What are the important features of type 2b VWD?

Answer 5

Associated with low platelets
May need platelet transfusion in pregnancy
Can’t receive DDAVP - worsens low platelets

Question 6

What are the important features of type 2N VWD?

Answer 6

Recessive inheritance

Associated with low factor 8

Question 7

What are the features of type 3 VWD?

Answer 7

Almost complete absence of VWF + low factor 8
Most rare and severe form
Does not respond to DDAVP
Can’t have epidural

Question 8

What are the risks to mum’s with VWD in pregnancy?

Answer 8

Bleeding - antepartum, primary and secondary PPH and during invasive procedures
Thrombocytopaenia in type 2b

Question 9

What are the risks to babies from mum’s with VWD?

Answer 9

Inheritance
Type 2 and 3 are managed as per haemophilia e.g. as if baby could get ECH, ICH e.g. avoid kiwi, fbs, fse, rotational and mid cavity forceps

Question 10

How should type 1 VWD patients be managed in labour?

Answer 10

If VWF activity >0.5 at Term, can have epidural and normal delivery

Question 11

How is VWF replaced pre invasive procedure?

Answer 11

If type 1 or type 2 (other than 2b) - first line is DDAVP unless contraindications

Question 12

What are the contraindications of DDAVP?

Answer 12

Type 2 B, type 3
PET
Uncontrolled hypertension, arterial disease
Unresponsive to DDAVP/ reducing responsiveness

Question 13

How is VWF increased if DDAVP can’t be used?

Answer 13

VWF concentrates which include vwf and factor VII

Question 14

What is the risk to the patient with VWF concentrates?

Answer 14

Risk of hep A and parvovirus infection

Question 15

How are patients with VWD managed in labour?

Answer 15

TXA
Monitoring and management of VWF activity and factor VIII levels (and platelets in type 2 disease)
IM and epidural should be avoided in <0.5
epidural should be avoided in type 3s
Type 2 and type 3 babies should be managed like severe haem A and B

Question 16

How are women with VWD managed postpartum?

Answer 16

1-2 weeks of TXA
3 day (SVD), 5 day (c/s instrumental) - monitoring/ management of VWF and VIII above 0.5
LWMH if indicated and >0.5

Question 17

How are neonates managed for mums with VWD?

Answer 17

cord blood testing - type 2 and 3 in particular
type 1 may be more appropriate to test at 6 months as likely to be negative
oral vit k if results not back for type 2 and 3
Cranial imaging may be necessary for type 3 babies