Pregnancy outcome in women with cystic fibrosis and poor pulmonary function

Abstract

Background: To investigate how poor pre-gestational pulmonary function influenced pregnancy outcome and clinical status evolution in women with cystic fibrosis. Methods: Pregnancies in women without lung transplantation with a first delivery reported to the French cystic fibrosis registry between 20 0 0 and 2012 were identified. Pregnancy outcomes and clinical trends (body mass index-BMI, and pulmonary function) over a 4-year follow-up in women with poor pregestational pulmonary function, defined as forced expiratory volume (FEV 1) ≤ 50%, were compared to those in women with FEV 1 ˃ 50%. Results: A total of 149 women had a first delivery and 36 (24.2%) of these had pre-gestational FEV 1 ≤ 50%. There was no significant difference in age or frequency of assisted conception between the 2 groups. The rate of cesarean section was significantly higher in women with FEV 1 ≤ 50% (43.7% vs. 21.1%, p = .01). The frequency of preterm birth did not differ significantly between the two groups, but median infant birthweight was significantly lower in women with FEV 1 ≤ 50% (2705 g; range: 650-3700 vs. 3044 g; range: 1590-3860, p = .003). Despite significantly lower FEV 1 and BMI the year before pregnancy for women with poor pulmonary function, the decline in these parameters during the study period did not differ significantly between the two groups. Conclusion: Poor pre-gestational pulmonary function in women with cystic fibrosis was associated with a higher rate of cesarean section and a clinically significant impact on fetal growth, but was not associated with more important pulmonary and nutritional decline over the study period.

Perinatal Outcomes in Cystic Fibrosis Women: Data From the Italian Cystic Fibrosis Registry.

“…In relation to the type of birth, it has been reported that 41.9% of CF women with a ppFEV1 of <50% [15] and 45.4% of those giving birth prematurely [13] undergo caesarean sections, whereas we found that a much higher proportion of cases (63.64%) were managed by means of a caesarean delivery. Our data show that there is no association between caesarean delivery and the severity of pre-conception respiratory function impairment, the age of the mother or her nutritional status by genotype, but there is a more significant association with a severe genotype than with the presence of at least one RF or VCC variant (p = 0.042).…”

“…A number of reports [5][6][7][8][9][10] have documented the effect of pregnancy on the clinical status of women with CF, but less is known about perinatal outcomes [11][12][13]. Other than the annual number of pregnancies, the American, British, Canadian and Australian CF registries provide no information in terms of gestational age or type of labour; only the French registry reports some data concerning perinatal outcomes [14,15].…”

The outcome of pregnancy in women with cystic fibrosis: a UK population‐based descriptive study

“…For the same reason, this study is unable to elucidate maternal or neonatal outcomes beyond the immediate postnatal period. Studies investigating longer‐term outcomes have reported a decline in lung function and a higher chance of death in the years after pregnancy in women with a lower pre‐pregnancy FEV 1 , 5,9 although a recent study of French registry data did not demonstrate a difference in decline in lung function over a 2‐year post‐pregnancy period in women with FEV 1 <50% compared with women who had FEV 1 >50% predicted 11 …”

“…Pre‐pregnancy lung function is often cited as the most important factor in predicting pregnancy outcomes for both mother and baby. Forced expiratory volume in 1 minute (FEV 1 ) of <60% predicted correlates with increased risk of premature delivery, 5–9 delivery by caesarean section 5,6 and adverse fetal outcomes such as low birthweight and perinatal death 6,10,11 . Guidelines for the management of pregnant women with CF were published in 2008 12 suggesting that an FEV 1 of <50% predicted should be considered an absolute contraindication to pregnancy.…”

“…The largest UK study pooled data from 15 CF centres presenting 72 pregnancies between 1966 and 1997 6 . More recent UK studies presented 15 pregnancies over an 8‐year period 13 and 48 pregnancies over 13 years, 5 and a recent study of the French CF registry analysed 149 pregnancies over a 12‐year period 11 …”