Pregnancy Outcomes in Women with Biventricular Circulation and a Systemic Right Ventricle: A Systematic Review

Background: Pregnancy in women with biventricular circulation and a systemic right ventricle (sRV) is considered high risk, with limited data available on pregnancy outcomes. This study aimed to investigate pregnancy outcomes in this population. Materials and Methods: A systematic review was conducted using four major electronic databases. Pregnant women with a complete transposition of great arteries (d-TGA) after an atrial switch operation or a congenitally corrected transposition of the great arteries (ccTGA) were included. Results: In total, 15 studies including 632 pregnancies in 415 women with an sRV and biventricular circulation were identified, of whom 299 (72%) had d-TGA and 116 (28%) ccTGA. Maternal mortality or cardiac transplantation occurred in 0.8% of pregnancies. The most frequent maternal complications were the worsening of systemic atrioventricular valve regurgitation [pooled estimate (PE): 16%, 95% CI: 5;26], the deterioration of sRV function (PE: 15%, 95% CI: 2;27), the worsening of the NYHA class (PE: 13%, 95% CI: 6;20), all-cause hospitalization (PE): 10%, 95% CI: 7;12), arrhythmias (PE: 8%, 95% CI: 5;11), and symptomatic heart failure (PE: 6%, 95% CI: 3;10). Stillbirth occurred in 0.7% of pregnancies and neonatal death in 0.4%. Small-for-gestational-age neonates were encountered in 36% (95% CI: 21;52) of pregnancies and preterm delivery in 22% (95% CI: 14;30). A subgroup analysis showed no significant difference in outcomes between women with d-TGA and those with ccTGA, except for the worsening of the NYHA class, which occurred more often in d-TGA (18%, 95% CI: 12;27 vs. 6%, 95% CI: 3;15, respectively, p = 0.03). Conclusions: Maternal and fetal/neonatal mortality are low among pregnant women with biventricular circulation and an sRV. However, significant maternal morbidity and poor neonatal outcomes are frequently encountered, rendering management in specialized centers imperative.