Pregnancy Outcome and Pulmonary Hypertension

Study Questions:

What are pregnancy outcomes in patients with pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD)?


This was a retrospective study utilizing questionnaires and chart reviews from seven French cardiac referral centers evaluating all pregnancies in patients with PAH/CHD from 1997-2015, including miscarriages (n = 2), elective terminations (n = 8), and completed (>20 weeks, n = 18) gestations.


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.


Due to the severity of maternal and neonatal complications, women with PAH/CHD should be counseled against pregnancy. If they elect to continue with pregnancy, care should be provided by a multidisciplinary, experienced team.


Pregnancy in patients with PAH/CHD continues to be high risk, especially for those with resting cyanosis. Pregnancy itself may adversely affect PAH progression. For women choosing pregnancy, many management questions remain, including optimal anticoagulation; anesthesia strategy; and safety, efficacy, and timing of initiation of advanced PAH therapy. Interestingly, most women in this study initially had normal biventricular function and most episodes of heart failure occurred postpartum, emphasizing the need for vigilance in this critical time.

Clinical Topics: Anticoagulation Management, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Valvular Heart Disease, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, Acute Heart Failure, Pulmonary Hypertension, Interventions and Structural Heart Disease, Hypertension

Keywords: Anesthesia, Antibiotic Prophylaxis, Anticoagulants, Cardiac Surgical Procedures, Cesarean Section, Cyanosis, Double Outlet Right Ventricle, Ductus Arteriosus, Patent, Endocarditis, Fetal Growth Retardation, Heart Defects, Congenital, Heart Failure, Heart Septal Defects, Atrial, Heart Septal Defects, Ventricular, Hypertension, Hypertension, Pulmonary, Postpartum Hemorrhage, Pregnancy, Pregnancy Outcome, Pulmonary Atresia, Thromboembolism, Transposition of Great Vessels, Truncus Arteriosus

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