Abstract
Congenital portal vein stenosis is a narrowing of the portal vein that may lead to liver cirrhosis. It is an abnormality that is usually discovered accidentally following investigation of a metabolic disturbance or intra-abdominal tumors in children or adults. Here, we describe the case of a 23-year-old pregnant woman with a medical history of congenital portal vein stenosis complicated by liver cirrhosis and portal vein hypertension. The patient presented at 36 weeks of gestation with generalized body edema. Following a normal vaginal delivery, significant postpartum hemorrhage occurred, which required multidisciplinary team involvement and intensive care unit admission. Ultimately, a clinical and hemodynamic response was noted. This case of a young female with these conditions illustrates the complexities involved in managing such pregnancies. Congenital vein stenosis is a rare condition that may be exacerbated by the physiological changes that already occur during pregnancy. Fetal and maternal complication risk increases, including intrauterine growth restriction, spontaneous abortions, and maternal death following hemodynamic compromise. Hence, advanced measures and clinical anticipation of complications in such patients will significantly enhance both maternal and fetal outcomes.