Management of childbirth in a patient with severe Factor V deficiency and congenital cardiomyopathy

Introduction: Therapeutic guidance regarding delivery in factor V (FV) deficient patients is scarce. Overall these patients are at increased risk of postpartum hemorrhage (PPH), especially when FV levels are very low. The preferred treatment in the peripartum period consists of administrating fresh frozen plasma (FFP), targeting a FV level of 15–25 IU/dL. Case report: We present the case of a 20-year old pregnant woman with a FV activity <1 IU/dL, but very limited parahemophilia symptoms. She was referred to our hospital because delivery of her first child had been complicated by acute lung edema (ALE) following administration of FFP. After the birth of her second child, she suffered from an important PPH, despite adequate FV substitution. Administration of six additional units of FFP resulted once more in ALE. Cardiologic work-up was warranted, and revealed a non-compaction cardiomyopathy. Six years later, despite negative advice regarding another pregnancy, this patient came again to our hospital bearing a third child. This time, prior to delivery, she was gradually given ten units of FFP together with diuretics, achieving a FV level of 26 IU/dL. Acute lung edema and PPH did not develop. Conclusions: When administering FFP, one should always remain aware of fluid overload. In our case, symptoms became apparent more rapidly because of a coexisting cardiomyopathy, but this does not need to be the case in every patient. When parahemophilia patients get older, mild preexisting heart failure may lead to similar situations when surgery with a high bleeding risk is required