Multidisciplinary Management of Pulmonary Arterial Hypertension in Pregnancy With Complex Congenital Heart Disease.

BACKGROUND: Pregnancy in individuals with pulmonary hypertension associated with adult congenital heart disease carries substantial maternal and fetal risk and is classified as modified World Health Organization 2.0 class IV when pulmonary arterial hypertension (PAH) is present. When pregnancy is continued, management requires individualized shared decision-making and expert multidisciplinary planning. CASE SUMMARY: We describe a case of a pregnant 24-year-old woman with complex congenital heart disease who presented with untreated PAH at initial cardio-obstetrics evaluation. The patient ultimately delivered a live-born infant. DISCUSSION: PAH in adults with congenital heart disease confers substantial maternal and fetal risk during pregnancy. TAKE-HOME MESSAGES: PAH carries a high risk during pregnancy with uncertain maternal and fetal outcomes. Multidisciplinary collaboration with close hemodynamic monitoring is needed for successful delivery planning, based on disease severity and local experience. Caring for PAH during pregnancy goes beyond delivery to ensure safe transition into the postpartum period.