Pulmonary Hypertension in Pregnancy

Abstract

The presence of pulmonary arterial hypertension (PAH) in pregnancy is rare and signifies a high-risk pregnancy. Although the majority of mothers have knowledge of their condition before pregnancy, approximately one-third of patients are diagnosed during pregnancy. Termination of pregnancy should be discussed, and is often advised; however, a significant proportion of patients will choose to proceed with the pregnancy despite increased maternal and fetal mortality. Currently, most pregnant patients receive advanced therapy for treatment of PAH, particularly prostacyclin analogues. Particular attention is paid to volume status and blood loss and there has been a major trend toward delivery by cesarean section under controlled conditions involving an expert multidisciplinary team. The time of greatest maternal risk is in the first month after delivery. Transplantation of these patients in the nonpregnant state may be considered when those with idiopathic pulmonary hypertension have poor functional status despite optimal therapy and their projected 2-year survival is less than 50%. For patients with Eisenmenger syndrome, severe symptoms and an unacceptable quality of life may lead to transplantation.

Endothelin-1 receptor antagonists in fetal development and pulmonary arterial hypertension

“…During normal pregnancy, the cardiac output increases due to a 50% increase in circulatory blood volume and an increase in heart rate of about 10-20 beats per minute [33]. To accommodate this increase in cardiac output without an increase in pulmonary artery pressure, dilatation and recruitment of additional pulmonary vessels is needed.…”

“…Pregnancy is contraindicated in female PAH patients [32], because cardiopulmonary changes during pregnancy and around delivery are associated with significant rates of disease progression and maternal death in PAH [33]. During normal pregnancy, the cardiac output increases due to a 50% increase in circulatory blood volume and an increase in heart rate of about 10-20 beats per minute [33].…”

“…Post-partum fluid shifts and a temporary deterioration of myocardial function are particular risks to female PAH patients in the first days after delivery [33,90]. If ERA's have a negative inotropic effect on the RV, this would be of particular importance during the third trimester of pregnancy, delivery and post-partum period.…”

“…Hemodynamic normalization after an uncomplicated delivery takes approximately six weeks [33,90,91]. Post-partum fluid shifts and a temporary deterioration of myocardial function are particular risks to female PAH patients in the first days after delivery [33,90].…”

Current Treatment Strategies, Guidelines and New Therapies

“…Thirty percent of pregnancies in women with PAH occur in patients who were not aware of their disease prior to pregnancy [ 177 ]. However, many patients diagnosed with PH are of childbearing age and early discussions regarding the risks of pregnancy are mandatory.…”