BACKGROUND: There is emerging evidence showing that maternal mortality in pregnant women with pulmonary artery hypertension, such as Eisenmenger syndrome (ES), is lower in recent years than historically described. In order to analyze recent outcomes, we retrospectively collected data of pregnancies in women with ES from 1997 to 2015.
METHODS: This multicenter study included women with ES, followed in 7 French referral centers for congenital heart disease. All pregnancies were included, including ectopic pregnancies, miscarriages and abortions. We collected data on maternal, obstetrical and neonatal outcome.
RESULTS: Twenty nine pregnancies in 18 women (25 ± 6 years old) with ES were managed during this period. There were 21 complete pregnancies (≥20 week gestation (WG)), 7 abortions, and 1 miscarriage. Six (32%) patients experienced severe cardiac events. These patients had lower saturation (79% vs. 89%) and were older. The most common cardiac complications after 20WG were heart failure (n = 4) and deep desaturation (n = 3).These heart failures were severe, requiring inotropic treatment (n = 3) or ventricular assistance device (n = 1), and lead to the only maternal death (mortality = 5%). Obstetrical complications occurred in 38% of pregnancies. Small gestational for age was diagnosed in 33% (7/21) and was statistically related to basal saturation level of the patient (p = 0.03), and to the maternal body mass index (p = 0.04). 12/21 (57%) pregnancies were delivered by cesarean section, with 7/12 for cardiac indications. The mean birth weight was 1824 ± 594 g at a mean gestational age of 34 ± 3WG. There was a high incidence of prematurity (57%), and no fetal or neonatal death.
CONCLUSIONS: Outcome of pregnancy in women with ES has improved with modern cardiologic and obstetrical management with a lower rate of maternal mortality. However, the severity of heart failure and the high rate of prematurity and SGA should still lead to counsel patients against pregnancy.
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© 2016 Published by Elsevier Inc.
