• Wentao Song, Shujuan Chu, Kai Yang, Xilong Sun, Haifa Xia, Chunli Mei, Yin Zhao, and Jing Wu.
• Department of Anesthesiology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
• J. Cardiothorac. Vasc. Anesth. 2022 Aug 1; 36 (8 Pt A): 2511-2517.

BackgroundSystemic lupus erythematosus (SLE) accounts for the largest portion of connective tissue disease-associated pulmonary arterial hypertension (PAH) in Asian countries, especially in China, and SLE-PAH poses multiple challenges during pregnancy and delivery. Patients with SLE-PAH tend to have lower survival rates and worse quality of life than other subgroups of PAH.Case PresentationPresented in this report is a 28-year-old primipara who suffered from SLE for 13 years and SLE-PAH for nine years. She had cardiac care throughout these years. She was admitted at 26 weeks of gestation for progressive dyspnea on exertion and her condition improved after a three-week PAH-targeted therapy consisting of prostacyclin and PDE-5 inhibitor. At 29 weeks of gestation, she was infected with influenza H1N1 and her clinical status deteriorated with increased dyspnea. After two weeks of influenza therapy and maximization of PAH therapy, a cesarean delivery was performed under epidural anesthesia at 31 weeks of gestation. She was discharged ten days after delivery. Although the targeted therapy for both PAH and SLE was readjusted after delivery and regular follow-up showed a gradual recovery and a stable condition, she still died suddenly at home 12 months after delivery. The child is healthy.ConclusionsSequential combination therapy of PAH and SLE and the structured perinatal management might lead to optimal short-term outcomes in the mother and fetus. Long-term outcomes in women with PAH who become pregnant are poor, with high rates of morbidity and mortality. Delivery strategies remain an important challenge for modern Pregnancy Heart Teams.Copyright © 2021. Published by Elsevier Inc.

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