Management of pulmonary arterial hypertension during pregnancy

ABSTRACT

Introduction

Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure >20 mmHg and pulmonary vascular resistance >2 Wood Units (WU) on right-heart catheterization. Pregnancy is generally contraindicated in PAH, it is associated with high maternal mortality. Despite current recommendations, the number of women with PAH wishing to become pregnant is increasing. Specialist care is essential for preconception counseling, and the management of pregnancy and delivery in such patients.

Areas covered

We cover the physiology of pregnancy, and its effects on the cardiovascular system in PAH. We also discuss optimal management based on available evidence and guidance.

Expert opinion

Pregnancy should be avoided in most patients with PAH. Counseling on appropriate contraception should be offered routinely. Education of women with childbearing potential is essential and should start at the time of diagnosis of PAH, or the time of transition from pediatric to adult services in patients developing PAH in childhood. Women wishing to become pregnant should receive individualized risk assessment and optimization of PAH therapies via a dedicated specialist pre-pregnancy counseling service, to minimize risk and improve outcomes. Pregnant PAH patients should receive expert multidisciplinary management in a PH center, including close monitoring and early initiation of therapies.

KEYWORDS:

• Pulmonary arterial hypertension
• cardiovascular disease and pregnancy
• congenital heart disease
• pulmonary arterial hypertension therapies
• pregnancy physiology

Article highlights

• Maternal mortality in PAH has decreased over the last few decades but remains significant.

• Pregnant women with PAH require expert multidisciplinary team care in a PH center.

• Close monitoring is required throughout pregnancy.

• Early delivery via cesarean section between 34 and 36 weeks with regional anesthesia is generally preferred. Birth plans should be made in accordance with maternal risk profiles and on an individualized basis. Fetal implications of preterm delivery should be considered.

• The postpartum period is associated with the highest mortality, hence post-delivery care should be provided in an intensive care environment.

Abbreviations

CHD	=	congenital heart disease
GA	=	general anaesthetic
mWHO	=	modified World Health Organisation
PAH	=	pulmonary arterial hypertension
PE	=	pulmonary embolism
PH	=	pulmonary hypertension
PVR	=	pulmonary vascular resistance
RV	=	right ventricle

Declaration of interest

K Krishnathasan received educational grants, personal fees, and non-financial support from Janssen-Cilag Limited. K Dimopoulos received non-financial support from Janssen-Cilag Limited; has been a consultant to and received grants and personal fees from Janssen-Cilag Limited, Pfizer, GlaxoSmithKline, and Bayer/MSD. A Constantine received educational grants, personal fees, and non-financial support from Janssen-Cilag Limited. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Correction Statement

This article has been republished with minor changes. These changes do not impact the academic content of the article.

Additional information

Funding

This paper was not funded.