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Management of Qualitative Plasminogen Activator Inhibitor-1 Deficiency in an Early Term Parturient
Abstract Number: T-63
Abstract Type: Case Report/Case Series
Plasminogen Activator Inhibitor-1 (PAI-1) deficiency has been previously described in Amish, European, and Asian populations. An autosomal recessive bleeding disorder, PAI-1 deficiency in the setting of pregnancy has been observed to be variable in its presentation.(1) A frameshift mutation in exon 4 of the PAI-1 gene has been identified as a cause for the disease; another mutation has been identified in exon 2. Current activity assays include zero as being within the range of normal, and thus lack sensitivity in providing a reliable diagnosis of PAI-1 deficiency. We present the peripartum overview of a patient with a clinical diagnosis of PAI-1 deficiency.
A 26 y/o G3P1011 was referred for anesthesia consultation at 26 weeks from the division of maternal fetal medicine. Her past medical history was significant for diagnosis of PAI-1 deficiency upon workup for menorrhagia as a teenager. Her obstetric history was significant for elective primary cesarean section under general endotracheal anesthesia. Postoperatively, she was placed on aminocaproic acid for 2 weeks. However, at 8 weeks, she encountered an episode of postpartum hemorrhage requiring further aminocaproic acid therapy.
Previous genetic analysis was negative for any known established mutations; PAI-1 activity levels were determined to be within the low-normal range during this current pregnancy. Antenatal thromboelastogram was significant for normal values during pregnancy. Given the patient's prior diagnosis and her presently inconclusive findings, the decision was made to perform a repeat cesarean under general anesthesia at 37 weeks. Immediately upon delivery, she received a dose of tranexamic acid. The patient tolerated the procedure well. She was subsequently discharged on an oral dose of tranexamic acid; there were no bleeding complications by the time of her 2 week postpartum visit.
We present our experience in the management of a patient with a history consistent with bleeding diathesis due to suspected PAI-1 deficiency. PAI-1 levels were obtained during this current pregnancy that were in the low-normal range. It is notable that increases in PAI-1 levels are considered part of the normal hemostatic changes of pregnancy.(2) Also during pregnancy, PAI-2 is secreted by the placenta. Collectively this may have potentially contributed to our within-range results on TEG. Regarding her history of postpartum hemorrhage, we theorize this may have coincided with a resumption of her menses and a decline in PAI-2 levels. PAI-2 levels have been demonstrated to remain in circulation up to 8 weeks postpartum.(2) We suspect a follow-up TEG at this timepoint may potentially reflect changes in her coagulation profile.*
1. Haemophilia. 2014, vol. 20, 407.
2. Semin Thromb Hemost. 2003, vol 29, 125.
*Patient is 3 weeks postpartum at time of abstract submission.