Pregnancy and pulmonary arterial hypertension: A clinical conundrum

Abstract

Pulmonary arterial hypertension (PAH) is a rare and devastating disease characterized by progressive increases in pulmonary arterial pressure and pulmonary vascular resistance which eventually leads to right ventricular failure and death. PAH inflicts most commonly women, majority of who are of childbearing age. Pregnancy in the setting of PAH is absolutely contraindicated due to high maternal fetal morbidity and guidelines do not exist for the management of such cases. A MEDLINE/PubMed search was performed identifying all relevant articles with "pulmonary arterial hypertension" and "pregnancy" in the title. Six case series were reviewed as well as our own center's experience outlined. Though there exists generalized treatment measures that are followed in such cases, management varies among different national centers as well an on an international level. At our center patients are managed using a multidisciplinary approach at a high risk obstetric center with preference for intravenous prostacyclin therapy. Women of child bearing age with possible signs and symptoms of PAH must be promptly diagnosed and managed expectantly with an emphasis on maternal-fetal safety.

Keywords: Congenital heart disease; Eisenmenger’s syndrome; High risk pregnancy; Pulmonary arterial hypertension; Pulmonary hypertension.