Management and Outcomes of Pregnancy in Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Background: Pregnant women with pulmonary arterial hypertension associated with congenital heart diseases (PAH-CHD) have a high incidence of mortality and adverse outcomes for mother and child. Methods: We retrospectively examined the treatment strategies and analyzed the outcomes of pregnancy in patients with pulmonary arterial hypertension managed at a single clinical hospital from 2009 to 2018. Results: Analysis of all 102 patients with PAH-CHD in pregnancy showed that maternal and newborn death from the disease was low(<3%, 3/102) compared to rates previously reported. Although patients with mild pulmonary hypertension can deliver safely, those with moderate to severe pulmonary artery pressure (PAP), and high functional class tend to have a high risk of heart failure. Medications were selectively administered to patients with more severe disease, and it was, therefore, challenging to make a universal statement on their benefit, but they appear having some benefits in improving birth outcomes for mother and child. While some treatments such as anticoagulant therapy during pregnancy, and oxytocin after delivery, did not improve the health outcome of pregnant women but seemed to provide some benefits to the newborns. Conclusion: Our retrospective analysis of existing clinical data provides preliminary results for further studies to formally evaluate the efficacy of clinical management of patients with pulmonary arterial hypertension.