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

Objective: To clarify the real-world outcomes in pregnant women with Eisenmenger syndrome (ES) in the new therapeutic era and provide a literature review.

Design: Retrospective case and literature review.

Setting: Tertiary referral hospital (The Second Xiangya Hospital of Central South University).

Sample: Thirteen women with ES delivered between 2011 and 2021.

Methods: Respective study and literature reviews.

Main outcomes measures: Maternal and neonatal mortality and morbidity.

Results: 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.

Conclusion: Our case series and literature review suggest that targeted drugs may be key to improving maternal mortality in ES.