Abstract： Pulmonary arterial hypertension (PAH) is an abnormal remodeling of the pulmonary vascular wall due to various causes, resulting in severe cardiovascular disease characterized by increased pulmonary vascular resistance and pressure. The mortality and morbidity of pregnant women with PAH are extremely high. This article reports a woman with severe PAH associated with connective tissue disease who developed cardiac arrest, PAH crisis, and right heart failure during her two consecutive pregnancies without regular prenatal examination. After multidisciplinary consultation and extracorporeal membrane oxygenation, effective cardiopulmonary support was timely, and the patient was finally discharged from the hospital in stable condition. After ten months of follow-up, the mother and child both had good outcomes. Although the mother and her child were survived, severe PAH is a contraindication for pregnancy due to its severely harmful effect on endangering maternal and fetal health.