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Pulmonary Hypertension

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Pregnancy and Congenital Heart Disease

Part of the book series: Congenital Heart Disease in Adolescents and Adults ((CHDAA))

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Abstract

The most common form of pulmonary hypertension (PH) in adult patients with congenital heart disease (ACDH) is pulmonary arterial hypertension (PAH) secondary to shunt lesions. The most severe form is the Eisenmenger’s syndrome with PAH, right to left shunt, and cyanosis. Pregnancy in PH or PAH is related to unacceptably high maternal morbidity and mortality. Neonatal outcome is also significantly compromised. In general, pregnancy is strongly discouraged in ACHD patients with PAH. In most cases, termination should be discussed and performed in an experienced tertiary care center. Women who would like to continue pregnancy need to be referred to a tertiary care center for follow-up and delivery. There tends to be a beneficial effect of advanced therapy for PAH, but strong data on safety and efficiency for pregnant women and fetus remain scarce. The mode of delivery is still under discussion. Both Caesarean section and vaginal delivery have their specific hemodynamic advantages and disadvantages. Expert anesthetic management and continuous monitoring during the peripartum are essential to keep complications as low as possible and to diagnose problems in a timely manner. In summary, the best management is to discourage pregnancy by an expert counseling team based on the risk scores of the WHO classification. As a consequence, the most effective and safe type of contraception has to be discussed.

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Abbreviations

BMPR2:

Bone morphogenetic protein receptor type 2

CS:

Caesarean section

EIF2AK4:

Eukaryotic translation initiation factor 2 alpha kinase 4

HIV:

Human immunodeficiency virus

LVEF:

Left ventricle ejection fraction

NYHA:

New York Heart Association

PAH:

Pulmonary arterial hypertension

PH:

Pulmonary hypertension

WHO:

World Health Organization

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Author information

Authors and Affiliations

  1. Congenital and Structural Cardiology, University Hospitals Leuven, Department of Cardiovascular Sciences, Catholic University Leuven, Leuven, Belgium

    Werner Budts MD, PhD

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  1. Werner Budts MD, PhD
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Correspondence to Werner Budts MD, PhD .

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Editors and Affiliations

  1. Erasmus MC University Medical Center, Rotterdam, Gelderland, The Netherlands

    Jolien W. Roos-Hesselink

  2. Chelsea and Westminster Hospital, London, United Kingdom

    Mark R. Johnson

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Budts, W. (2017). Pulmonary Hypertension. In: Roos-Hesselink, J., Johnson, M. (eds) Pregnancy and Congenital Heart Disease. Congenital Heart Disease in Adolescents and Adults. Springer, Cham. https://doi.org/10.1007/978-3-319-38913-4_17

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  • DOI: https://doi.org/10.1007/978-3-319-38913-4_17

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