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Pregnancy is usually contraindicated in patients with pulmonary hypertension (PH). Risk factors associated with the outcome of this rare disease have not been specifically explored before.Medical records were retrospectively reviewed to identify patients with coexisting PH and pregnancy or delivery at Peking Union Medical College Hospital between January 2009 and June 2018. Demographics, characteristics of PH and pregnancy, management and outcomes were analyzed.Thirty-six pregnant women with PH were identified, including 30 cases in WHO group 1, 5 cases of group 2 and 1 case of group 4. Median pregnancy duration was 24 weeks. The overall maternal mortality rate was 8.3% (3/36), and the late fetal mortality was 31.6% (6/19). Pulmonary vascular-targeted medications were used in 17 of 26 patients with moderate or severe PH, but in none with mild PH. Maternal mortality was 2/15, 1/11, and 0 among women with severe, moderate, and mild PH, respectively. All deaths reported to be diagnosed of PH after pregnancy, and have New York Heart Association (NYHA) grades II to IV. Cesarean section was performed in 22 patients, and mortality was 3/16 among women receiving cesarean section with general anesthesia.Maternal mortality is associated with PH classification, severity of PH, delayed diagnosis of PH, and NYHA classification. Regional anesthesia seems superior to general anesthesia for cesarean section.