Successful Eisenmenger syndrome‐targeted drug therapy in pregnant women: a case series and literature review

To clarify the real-world outcomes in pregnant women with Eisenmenger syndrome (ES) in the new therapeutic era and provide a literature review.Retrospective case and literature review.Tertiary referral hospital (The Second Xiangya Hospital of Central South University).Thirteen women with ES delivered between 2011 and 2021.Respective study and literature reviews.Maternal and neonatal mortality and morbidity.12/13 (92%) pregnant women were treated with targeted drugs. 9/13 (69%) of patients had heart failure, but no maternal deaths occurred. 12/13 (92%) of women chose caesarean delivery. One pregnant woman gave birth at 37+1 weeks, and the remaining 12 (92%) patients had preterm birth. 10/13 (77%) women gave birth to live infants, of which 9/10 (90%) were low birthweight infants with a mean birthweight of 1575 g. The infant mortality rate was 1/10 (10%). Cardiac functional class improved during pregnancy, probably due to therapy; 11/13 (85%) of the pregnant women were in cardiac functional level III/IV at admission and 12 (92%) were in cardiac functional class II/III at discharge. Our literature review identified 72 cases of pregnancy with ES from 11 studies, which were characterised by a low rate of targeted drug use (28%) and a high maternal mortality rate of 24% in the perinatal period.Our case series and literature review suggest that targeted drugs may be key to improving maternal mortality in ES.