Results:

Twenty-eight pregnancies from 20 patients (ages 26 ± 6 years) were included. Ventricular septal defect (VSD) was the most common CHD; other lesions included atrial septal defect (ASD), pulmonary atresia/VSD with aortopulmonary collaterals, patent ductus arteriosus, double outlet right ventricle, truncus arteriosus, atrioventricular canal, congenitally corrected transposition of the great arteries, and single ventricles, in order of frequency, with seven having had palliative surgery. Seventeen had Eisenmenger’s (PAH group 1) and three had segmental PAH from pulmonary atresia/VSD with collaterals (PAH group 5) (see Simonneau G, et al., Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013;62:25_S). Of the 18 completed pregnancies, five had worsening hypoxemia and four had severe heart failure (three postpartum), requiring inotropes and extracorporeal membrane oxygenation, with one postpartum maternal death. Four had postpartum hemorrhage (two received anticoagulation). Gestational age ranged from 28-38 weeks (78% preterm), with intrauterine growth retardation in 39% (mean birth weight 1.7 kg), but no stillbirths. There were no thromboembolisms or endocarditis, with six receiving anticoagulation and seven receiving antibiotic prophylaxis, respectively. Twelve were delivered by caesarean section (10 for obstetric reasons). Patients were followed for a median duration of 9 years, two died >4 years after delivery, with one third experiencing worsening in New York Heart Association functional class.