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Pulmonary Vasodilators in Patients with Pulmonary Arterial Hypertension Related to Congenital Heart Disease

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Pulmonary Hypertension in Adult Congenital Heart Disease

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

Abstract

The presence of pulmonary arterial hypertension (PAH) increases morbidity and mortality in patients with congenital heart disease (CHD). PAH associated with CHD is a heterogeneous condition, depending on the type of the underlying defect and previous repair strategies. There is growing evidence of the benefits of pulmonary vasodilators in this population, but, despite recent advances, mortality remains relatively high. Currently, there are limited data to guide the use of pulmonary vasodilators in this population. Furthermore, particular subgroups such as patients with Fontan circulation or “segmental” pulmonary hypertension are challenging to manage and even more challenging to treat. The current chapter reviews the different therapies for PAH in this growing and challenging population.

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Abbreviations

ASD:

atrial septal defect

CTEPH:

chronic thromboembolic pulmonary hypertension

PAH:

pulmonary arterial hypertension

PAH–CHD:

pulmonary arterial hypertension associated with congenital heart disease

PAH–CTD:

pulmonary arterial hypertension associated with connective tissue disease

PAH–HIV:

pulmonary arterial hypertension associated with human inmunodeficiencyvirus

PDA:

patent ductus arteriosus

VSD:

ventricular septal defect

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Author information

Authors and Affiliations

  1. NIHR Cardiovascular BRU, Royal Brompton Hospital and the National Heart and Lung Institute, Imperial College, London, UK

    Rafael Alonso-Gonzalez M.D., M.Sc.

  2. Pulmonary Hypertension and Adult Congenital Heart Disease Unit, Hospital 12 de Octubre, Madrid, Spain

    Pilar Escribano-Subías M.D., Ph.D.

Authors
  1. Rafael Alonso-Gonzalez M.D., M.Sc.
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  2. Pilar Escribano-Subías M.D., Ph.D.
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Corresponding author

Correspondence to Rafael Alonso-Gonzalez M.D., M.Sc. .

Editor information

Editors and Affiliations

  1. Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College, London, United Kingdom

    Konstantinos Dimopoulos

  2. University Hospital, Münster, Germany

    Gerhard-Paul Diller

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Alonso-Gonzalez, R., Escribano-Subías, P. (2017). Pulmonary Vasodilators in Patients with Pulmonary Arterial Hypertension Related to Congenital Heart Disease. In: Dimopoulos, K., Diller, GP. (eds) Pulmonary Hypertension in Adult Congenital Heart Disease. Congenital Heart Disease in Adolescents and Adults. Springer, Cham. https://doi.org/10.1007/978-3-319-46028-4_16

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  • DOI: https://doi.org/10.1007/978-3-319-46028-4_16

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  • Online ISBN: 978-3-319-46028-4

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