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Post-capillary Pulmonary Hypertension in ACHD

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Pulmonary Hypertension in Adult Congenital Heart Disease

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

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Abstract

Pulmonary hypertension (PH) is a haemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure at rest ⩾ 25 mmHg as assessed by right heart catheterization. Haemodynamically PH can be further distinguished into pre-capillary PH depending on the presence of a raised left atrial or pulmonary wedge pressure. Post-capillary PH is common in patients with congenital heart disease, in whom systemic ventricular dysfunction, valve disease, outflow tract obstruction or pulmonary venous stenosis can lead to a rise in post-capillary pressures. In this chapter, we discuss the pathogenesis, impact and management of postcapillary PH in congenital heart disease.

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Abbreviations

ACHD:

adult congenital heart disease

CHD:

congenital heart disease

ccTGA:

congenitally corrected transposition of the great arteries

Cpc-PH:

combined post-capillary and pre-capillary PH

DPG:

diastolic pulmonary pressure gradient

ECG:

electrocardiography

HF:

heart failure

HFrEF:

heart failure with reduced ejection fraction

HFpEF:

heart failure with preserved ejection fraction

Ipc-PH:

isolated post-capillary PH

LA:

left atrium

LHD:

left heart disease

LV:

left ventricle

LVAD:

left ventricle assist device

LVEDP:

left ventricular end-diastolic pressure

LVEF:

left ventricular ejection fraction

mPAP:

mean pulmonary arterial pressure

PAH:

pulmonary arterial hypertension

PH:

pulmonary hypertension

PAP:

pulmonary arterial pressure

PAWP:

pulmonary artery wedge pressure

PVR:

pulmonary vascular resistance

RHC:

right heart catheterization

SVR:

systemic vascular resistance

TGA:

transposition of the great arteries

TPG:

trans-pulmonary pressure gradient

WHO:

World Health Organization

WU:

Wood units

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Authors and Affiliations

  1. Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Albert-Schweitzer-Campus 1, Münster, 48149, Germany

    Margarita Brida & Gerhard-Paul Diller

  2. National Heart and Lung Institute, Imperial College London, London, UK

    Gerhard-Paul Diller

Authors
  1. Margarita Brida
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  2. Gerhard-Paul Diller
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Correspondence to Gerhard-Paul Diller .

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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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Brida, M., Diller, GP. (2017). Post-capillary Pulmonary Hypertension in ACHD. 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_8

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  • DOI: https://doi.org/10.1007/978-3-319-46028-4_8

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