Pregnancy outcomes in patients with pulmonary arterial hypertension associated with congenital heart disease

OBJECTIVE: There is growing evidence that maternal mortality in pregnant women with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is lower than that in available data. In order to evaluate this hypothesis, we collected data of pregnancies in women with PAH-CHD. METHODS: Women with PAH-CHD followed in seven French referral centres were retrospectively included from 1997 to 2015. All pregnancies were recorded. We collected data on maternal, obstetrical and neonatal outcomes. RESULTS: 28 pregnancies in 20 women (26±6 years old) with PAH-CHD were managed during this period. There were 18 complete pregnancies (≥20 weeks' gestation (WG)), 8 abortions and 2 miscarriages. Six (33%, 95% CI (11.9 to 54.3)) patients experienced severe cardiac events. The concerned women had lower resting oxygen saturation (79.6±4.1% vs 89.3±3.8%, p<0.01). The most common cardiac complications during the complete pregnancies were heart failure (n=4) and severe hypoxaemia (n=5). Heart failure was overall severe, requiring inotropic treatment in three patients, mechanical circulatory support in one and led to one maternal death (mortality=5.0% 95% CI (0.1 to 24.9)). Obstetrical complications occurred in 25% of pregnancies. Small for gestational age was diagnosed in 39% (7/18) of fetuses. 12/18 (67%) pregnancies were delivered by caesarean section, of which 10 in emergency for obstetrical reason. Prematurity was frequent (78%), but no neonatal death occurred. CONCLUSIONS: Outcome of pregnancy in women with PAH-CHD is better than previously reported, with only 5% maternal mortality in our cohort. However, because of the severity of heart failure and the high rate of neonatal complications, patients should still be advised against pregnancy.