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

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. 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. 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, = 0.03). 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.