Pulmonary arterial hypertension and pregnancy

Abstract

Pulmonary arterial hypertension (PAH) is a disease of the small pulmonary arteries that is characterised by vascular proliferation and remodelling. It results in a progressive increase in pulmonary vascular resistance and, ultimately, right ventricular failure and death. Despite recent major improvements in symptomatic treatments, no current treatment cures this devastating condition. Significant advances in the treatment of PAH have been achieved over the past decades. Given the high mortality rate of patients with PAH, pregnancy is not recommended. Patients who do become pregnant and wish to carry on with the pregnancy are treated with PDE-5 inhibitors, prostanoids, and supplemental therapy as needed. Other treatments such as endothelin receptor antagonists are contraindicated because they cause foetal malformations.