Maternal and neonatal outcomes in pregnancies with cystic fibrosis after lung transplantation or during elexacaftor/tezacaftor/ivacaftor (ETI) therapy: a single-center descriptive cohort.

BACKGROUND

Advances in cystic fibrosis (CF) therapy, particularly CFTR modulators such as elexacaftor/tezacaftor/ivacaftor (ETI), are reshaping reproductive outcomes for women with advanced CF. Historically, lung transplantation (LT) was the only means to achieve sufficient respiratory stability for pregnancy. This study provides real-world clinical data describing pregnancies in women with CF after LT and during ETI therapy.

METHODS

We conducted a retrospective descriptive study of all pregnancies resulting in live births among women with CF followed at the Nantes University Hospital CF reference center between January 1, 2000 and August 31, 2024. Maternal characteristics, obstetric complications, and neonatal outcomes were collected for pregnancies after LT or under ETI treatment. Analyses were descriptive, with no statistical comparisons between groups due to small sample size and inherent clinical differences. Descriptive values from the 2021 French National Perinatal Survey are reported alongside our results for context only.

RESULTS

Among 95 pregnancies in 46 women, 56 resulted in live births, including 7 after LT and 10 during ETI therapy. In the LT cohort, pre-existing comorbidities were frequent: cystic fibrosis-related diabetes (CFRD) (100%), chronic hypertension (50%), and moderate renal impairment (83.3%). Preterm delivery occurred in 57.1% of pregnancies and intensive care unit admission in 42.9%. In ETI-exposed pregnancies, no gestational hypertension or preeclampsia was identified, and no woman required intensive care admission or delivered before 34 weeks. No congenital anomaly was observed in either group. Median gestational age at birth was 38 weeks + 3 days and median birth weight 3190 g in the ETI group, versus 35 weeks + 4 days and 1980 g in the LT group; these values are reported descriptively and were not compared statistically.

CONCLUSIONS

In this real-world cohort, ETI-exposed pregnancies were associated with reassuring maternal and neonatal outcomes, while pregnancies after LT were characterized by frequent transplant-related and vascular morbidity; the two groups were described separately and were not formally compared. These findings support the need for multidisciplinary follow-up and prospective registries to evaluate maternal and pediatric outcomes in the CFTR modulator era.