Management of patients with pulmonary arterial hypertension due to congenital heart disease: recent advances and future directions

Abstract

Pulmonary arterial hypertension is a serious complication of adult congenital heart disease associated with systemic-to-pulmonary shunts. Although early shunt closure restricts development of pulmonary arterial hypertension, patients remain at risk even after repair. The development of pulmonary arterial hypertension is associated with a markedly increased morbidity and mortality. It is important to identify patients with a poor prognosis using disease specific markers. Echocardiography and biomarkers arise as practical tools to determine the risk of mortality. Although pulmonary arterial hypertension cannot be cured, four classes of disease-targeting therapies are currently available and several promising therapies are being studied. There is a shift in drug studies towards more clinically relevant endpoints such as time to clinical worsening and morbidity and mortality events.

Keywords:

• closed defect
• congenital heart disease
• management
• pulmonary arterial hypertension
• predictors
• surgery
• therapy

Acknowledgements

The work performed for this review was carried out in the context of the Parelsnoer Institute (PSI). PSI is part of and funded by the Dutch Federation of University Medical Centers.

ORCID

Ilja M. Blok http://orcid.org/0000-0001-9982-1762