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Pulmonary Arterial Hypertension in Patients with Previous Reparative Surgery

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Book cover Pulmonary Hypertension in Adult Congenital Heart Disease

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

Abstract

Pulmonary arterial hypertension (PAH) is a frequently diagnosed complication in patients with congenital heart disease (CHD). The majority of the cases involve patients with nonrestrictive congenital cardiac shunts with unprotected pulmonary blood flow. Exposure of the pulmonary vasculature to increased blood flow and pressure results in vascular remodelling and eventually established pulmonary vascular disease. Despite advances in the understanding of the pulmonary vascular disease, advances in surgical repair of congenital heart disease and the development of drug therapies, PAH still occurs after successful corrective surgery in patients with CHD and results in significant morbidity and mortality. In this chapter, we discuss the pathogenesis, presentation, natural history and management of PAH in patients with previous surgical or percutaneous repair of CHD.

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Abbreviations

ASD:

Atrial septal defect

AVSD:

Atrioventricular septal defect

BMPR-2:

Bone morphogenetic protein receptor type 2

CHD:

Congenital heart disease

CTD:

Connective tissue disease

ERA:

Endothelin receptor antagonist

ES:

Eisenmenger syndrome

FC:

Functional class

iPAH:

Idiopathic pulmonary arterial hypertension

LHD:

Left heart disease

mPAP:

Mean pulmonary artery pressure

NT-proBNP:

N-terminal prohormone of brain natriuretic peptide

PAH:

Pulmonary arterial hypertension

PAH–CHD:

Pulmonary arterial hypertension in congenital heart disease

PDE5:

Phosphodiesterase 5

PGs:

Prostaglandins

PVR:

Pulmonary vascular resistance

PVRI:

Pulmonary vascular resistance indexed

RCT:

Randomized control trials

RV:

Right ventricle

TGA:

Transposition of great arteries

VSD:

Ventricular septal defect

WHO:

World Health Organization

6MWT:

6-min walk test

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Author information

Authors and Affiliations

  1. Great Ormond Street Hospital, London, WC1N 3JH, UK

    Shahin Moledina

  2. Department of Paediatric Cardiology-Pulmonary Hypertension, Great Ormond Street Hospital, London, WC1N 3JH, UK

    Paraskevi Theocharis

Authors
  1. Shahin Moledina
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  2. Paraskevi Theocharis
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Corresponding author

Correspondence to Shahin Moledina .

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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Moledina, S., Theocharis, P. (2017). Pulmonary Arterial Hypertension in Patients with Previous Reparative Surgery. 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_5

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  • DOI: https://doi.org/10.1007/978-3-319-46028-4_5

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-46026-0

  • Online ISBN: 978-3-319-46028-4

  • eBook Packages: MedicineMedicine (R0)

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