Purpose of review This study aims to describe the pathophysiology of pregnancy in pulmonary hypertension (PH) and review recent literature on maternal and fetal outcomes. Recent findings There is an increasing number of pregnant women with PH. Maternal mortality in pulmonary arterial hypertension (PAH) ranges from 9 to 25%, most commonly from heart failure and arrythmias. The highest risk of death is peri-partum and post-partum. Fetal/neonatal morbidity and mortality are also substantial. There are high rates of prematurity, intrauterine growth retardation, and preeclampsia. Women should be referred to expert centers for management. Combination PAH therapy with parenteral prostacyclin and a phosphodiesterase type V inhibitor is recommended. Induced vaginal delivery is preferred, except in cases of severe heart failure or obstetric indications for cesarean section. Summary Despite advances in management, pregnancy in PAH remains a high-risk condition and should be prevented.
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