Assessment of obstetric characteristics and outcomes associated with pregnancy with Turner syndrome

Abstract

To assess national-level trends, characteristics, and outcomes of pregnancies with Turner syndrome in the United States. Cross-sectional study. The Healthcare Cost and Utilization Project's National Inpatient Sample. 17,865,495 hospital deliveries from 2016-2020. A diagnosis of Turner syndrome, identified according to the World Health Organization's International Classification of Disease 10th revision code of Q96. Obstetrics outcomes related to Turner syndrome, assessed with inverse probability of treatment weighting cohort and multivariable binary logistic regression modeling. Prevalence of pregnant patients with Turner syndrome was 7.0 per 100,000 deliveries (one in 14,235). Number of hospital deliveries with patients who have a diagnosis of Turner syndrome increased from 5.0 to 11.7 per 100,000 deliveries during the study period (adjusted-odds ratio [aOR] for 2020 vs 2016, 2.18, 95% confidence interval [CI] 1.83-2.60). Pregnant patients with Turner syndrome were more likely to have a diagnosis of pregestational hypertension (4.8% vs 2.8%, aOR 1.65, 95%CI 1.26-2.15), uterine anomaly (1.6% vs 0.4%, aOR 3.01 95%CI 1.93-4.69), and prior pregnancy losses (1.6% vs 0.3%, aOR 4.70, 95%CI 3.01-7.32) compared to those without Turner syndrome. For the index obstetric characteristics, Turner syndrome was associated with increased risk of intrauterine fetal demise (10.9% vs 0.7%, aOR 8.40, 95%CI 5.30-13.30), intrauterine growth restriction (8.5% vs 3.5%, aOR 2.11, 95%CI 1.48-2.99), and placenta accreta spectrum (aOR 3.63, 95%CI 1.20-10.97). For delivery outcome, pregnant patients with Turner syndrome were more likely to undergo cesarean delivery (41.6% vs 32.3%, aOR 1.53, 95%CI 1.26-1.87). Moreover, the odds of peri-viable delivery (22-25 weeks: 6.1% vs 0.4%, aOR 5.88, 95%CI 3.47-9.98) and pre-viable delivery (<22 weeks: 3.3% vs 0.3%, aOR 2.87, 95%CI 1.45-5.69) were increased compared to those without Turner syndrome. The results of contemporaneous, nationwide assessment in the United States suggests that although pregnancy with Turner syndrome is uncommon this may represent a high-risk group, particularly for intrauterine fetal demise and peri-viable delivery. Establishing society-based approach for preconception counseling and antenatal follow-up would be clinically compelling.