Role of Meticulous Observation: Successful Pregnancy in a 30-Year-Old Woman with Severe Pulmonary Hypertension

摘要

Pregnancy is strongly discouraged in patients with pulmonary arterial hypertension (PAH). Herein, we report a successful delivery in a woman with PAH using a multidisciplinary approach. A 30-year-old pregnant woman with PAH was referred to us with a chief complaint of dyspnea. She was advised to terminate her pregnancy but she refused to do so despite several recommendations by healthcare professionals. She was scheduled for treatment with iloprost (brand name: Ilomedin) and heparin infusions for 3–4 days at 20-day intervals. She spent her last month of pregnancy in a hospital under close observation and received iloprost infusion. She underwent a successful cesarean section under general anesthesia at week 36 of gestation. Iloprost administration was continued for one week after delivery and was changed to bosentan after that. Meanwhile, heparin infusion was substituted by warfarin. However, treatment with bosentan led to a temporary interruption in breastfeeding. A few days later, she presented with severe dyspnea and pulmonary artery pressure of 110 mmHg. Treatment was restarted with iloprost, followed by stabilization with bosentan. A successful delivery was achieved in this situation by meticulous observation and aggressive treatment targeting PAH, along with long-term hospital stay and multidisciplinary management. Severe PAH is regarded as a contraindication to pregnancy. While physicians strongly recommend termination of pregnancy in such patients, some of them might refuse and insist on delivery of the baby. Similar pregnant cases with potential delivery are recommended to be evaluated for effective management of this condition.