General principle and modalities of management of pregnancy in congenital dysfibrinogenemia: case 5
| Preconception counseling • Provide adequate information on potential clinical complications and implications of genetic screening. • Discuss pregnancy management. |
| Antenatal management • Quarterly assessment of fibrinogen activity level. • Systematic monitoring of fetal growth. • Fibrinogen supplementation targeting a fibrinogen activity trough level >1 to 1.5 g/L in cases with vaginal bleeding. |
| Labor and delivery • Fibrinogen supplementation targeting a fibrinogen activity peak level >1.5 g/L in cases with neuraxial analgesia. • Avoid invasive fetal procedures and forceps- or vacuum-assisted delivery. |
| Postpartum management • Monitoring of fibrinogen activity level. • Early fibrinogen supplementation and an antifibrinolytic drug in case of postpartum hemorrhage. • Thromboprophylaxis according to the same recommendation as for the general population, favoring a mechanical thromboprophylaxis whenever possible in patients with type 3A CD. • For patients with type 3B dysfibrinogenemia, adopt the same recommendations as for the general population, favoring pharmacological thromboprophylaxis whenever possible. |
| Preconception counseling • Provide adequate information on potential clinical complications and implications of genetic screening. • Discuss pregnancy management. |
| Antenatal management • Quarterly assessment of fibrinogen activity level. • Systematic monitoring of fetal growth. • Fibrinogen supplementation targeting a fibrinogen activity trough level >1 to 1.5 g/L in cases with vaginal bleeding. |
| Labor and delivery • Fibrinogen supplementation targeting a fibrinogen activity peak level >1.5 g/L in cases with neuraxial analgesia. • Avoid invasive fetal procedures and forceps- or vacuum-assisted delivery. |
| Postpartum management • Monitoring of fibrinogen activity level. • Early fibrinogen supplementation and an antifibrinolytic drug in case of postpartum hemorrhage. • Thromboprophylaxis according to the same recommendation as for the general population, favoring a mechanical thromboprophylaxis whenever possible in patients with type 3A CD. • For patients with type 3B dysfibrinogenemia, adopt the same recommendations as for the general population, favoring pharmacological thromboprophylaxis whenever possible. |