Abstract
The mortality rate of pulmonary hypertension in pregnancy is 25%-56%. Pulmonary arterial hypertension is the highest incidence among this group, especially in young women. Despite clear recommendation of pregnancy avoidance, certain groups of patients are initially diagnosed during the gestational age step into the third trimester. While the presence of right ventricular failure in early gestation is usually trivial, it can be more severe in the late trimester. Current evidence shows no consensus in the management and serious precautions for each stage of the pre-, peri- and post-partum periods of this specific group. Pulmonary hypertension-targeted drugs, mode of delivery, type of anesthesia, and some avoidances should be planned among a multidisciplinary team to enhance maternal and fetal survival opportunities. Sudden circulatory collapse from cardiac decompensation during the peri- and post-partum phases is detrimental, and mechanical support such as extracorporeal membrane oxygenation should be considered for mitigating hemodynamics and extending cardiac recovery time. Our review aims to explain the pathophysiology of pulmonary arterial hypertension and summarize the current evidence for critical management and precautions in each stage of pregnancy.