Abstract：
Women with hereditary fibrinogen disorders (HFDs) seem to be at an increased risk of adverse obstetrical outcomes, but epidemiologic data are limited.
We aimed to determine the prevalence of pregnancy complications; the modalities and management of delivery; and the postpartum events in women with hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia.
We conducted a retrospective and prospective multicentric international study.
A total of 425 pregnancies were investigated from 159 women (49, 95, and 15 cases of hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia, respectively). Overall, only 55 (12.9%) pregnancies resulted in an early miscarriage, 3 (0.7%) resulted in a late miscarriage, and 4 (0.9%) resulted in an intrauterine fetal death. The prevalence of live birth was similar among the types of HFDs (P = .31). Obstetrical complications were observed in 54 (17.3%) live birth pregnancies, including vaginal bleeding (14, 4.4%), retroplacental hematoma (13, 4.1%), and thrombosis (4, 1.3%). Most deliveries were spontaneous (218, 74.1%) with a vaginal noninstrumental delivery (195, 63.3%). A neuraxial anesthesia was performed in 116 (40.4%) pregnancies, whereas general or no anesthesia was performed in 71 (16.6%) and 129 (44.9%) pregnancies, respectively. A fibrinogen infusion was administered in 28 (8.9%) deliveries. Postpartum hemorrhages were observed in 62 (19.9%) pregnancies. Postpartum venous thrombotic events occurred in 5 (1.6%) pregnancies. Women with hypofibrinogenemia were at an increased risk of bleeding during the pregnancy (P = .04).
Compared with European epidemiologic data, we did not observe a greater frequency of miscarriage, while retroplacental hematoma, postpartum hemorrhage, and thrombosis were more frequent. Delivery was often performed without locoregional anesthesia. Our findings highlight the urgent need for guidance on the management of pregnancy in HFDs.
We aimed to determine the prevalence of pregnancy complications; the modalities and management of delivery; and the postpartum events in women with hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia.
We conducted a retrospective and prospective multicentric international study.
A total of 425 pregnancies were investigated from 159 women (49, 95, and 15 cases of hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia, respectively). Overall, only 55 (12.9%) pregnancies resulted in an early miscarriage, 3 (0.7%) resulted in a late miscarriage, and 4 (0.9%) resulted in an intrauterine fetal death. The prevalence of live birth was similar among the types of HFDs (P = .31). Obstetrical complications were observed in 54 (17.3%) live birth pregnancies, including vaginal bleeding (14, 4.4%), retroplacental hematoma (13, 4.1%), and thrombosis (4, 1.3%). Most deliveries were spontaneous (218, 74.1%) with a vaginal noninstrumental delivery (195, 63.3%). A neuraxial anesthesia was performed in 116 (40.4%) pregnancies, whereas general or no anesthesia was performed in 71 (16.6%) and 129 (44.9%) pregnancies, respectively. A fibrinogen infusion was administered in 28 (8.9%) deliveries. Postpartum hemorrhages were observed in 62 (19.9%) pregnancies. Postpartum venous thrombotic events occurred in 5 (1.6%) pregnancies. Women with hypofibrinogenemia were at an increased risk of bleeding during the pregnancy (P = .04).
Compared with European epidemiologic data, we did not observe a greater frequency of miscarriage, while retroplacental hematoma, postpartum hemorrhage, and thrombosis were more frequent. Delivery was often performed without locoregional anesthesia. Our findings highlight the urgent need for guidance on the management of pregnancy in HFDs.
摘要：
背景：患有遗传性纤维蛋白原紊乱（HFDs）的女性似乎在产科不良结局的风险增加，但流行病学数据有限患者/方法：我们进行了一项回顾性和前瞻性国际研究，以确定妊娠并发症的患病率，交付的方式和管理，和产后事件。
结果：从159名妇女中调查了425例怀孕（49例低纤维蛋白原血症，95纤维蛋白原异常血症，15低纤维蛋白原血症)。总的来说,只有55例（12.9%）怀孕导致早期流产，晚期流产3例（0.7％），宫内胎儿死亡4例（0.9％）。不同类型HFD的活产率相似(p=0.31)。在54例（17.3％）的活产妊娠中观察到产科并发症，包括阴道出血（14，4.4%），胎盘后血肿（13，4.1%），和血栓形成（4，1.3%）。大多数56例分娩是自发的（218，74.1％），而阴道非器械分娩（195，63.3％）。在116例（40.4%）妊娠中进行了神经轴麻醉，71例（16.6%）和129例（44.9%）全身麻醉或无麻醉，分别。在28例（8.9％）分娩中进行了纤维蛋白原输注。在62例（19.9％）的妊娠中观察到产后出血。产后静脉血栓事件发生在5例（1.6％）妊娠中。低纤维蛋白原血症的女性在怀孕期间更有出血的风险(p=0.04)。
结论：与欧洲流行病学数据相比，我们没有观察到更高的流产频率，而胎盘后血肿，产后出血和血栓形成更为频繁。通常在没有局部麻醉的情况下进行分娩。我们的发现强调了迫切需要指导HFDs的妊娠管理。
结果：从159名妇女中调查了425例怀孕（49例低纤维蛋白原血症，95纤维蛋白原异常血症，15低纤维蛋白原血症)。总的来说,只有55例（12.9%）怀孕导致早期流产，晚期流产3例（0.7％），宫内胎儿死亡4例（0.9％）。不同类型HFD的活产率相似(p=0.31)。在54例（17.3％）的活产妊娠中观察到产科并发症，包括阴道出血（14，4.4%），胎盘后血肿（13，4.1%），和血栓形成（4，1.3%）。大多数56例分娩是自发的（218，74.1％），而阴道非器械分娩（195，63.3％）。在116例（40.4%）妊娠中进行了神经轴麻醉，71例（16.6%）和129例（44.9%）全身麻醉或无麻醉，分别。在28例（8.9％）分娩中进行了纤维蛋白原输注。在62例（19.9％）的妊娠中观察到产后出血。产后静脉血栓事件发生在5例（1.6％）妊娠中。低纤维蛋白原血症的女性在怀孕期间更有出血的风险(p=0.04)。
结论：与欧洲流行病学数据相比，我们没有观察到更高的流产频率，而胎盘后血肿，产后出血和血栓形成更为频繁。通常在没有局部麻醉的情况下进行分娩。我们的发现强调了迫切需要指导HFDs的妊娠管理。
