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Pulmonary Arterial Hypertension

  • Despina Ntiloudi
  • George Giannakoulas
Chapter
Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)

Abstract

Pulmonary arterial hypertension (PAH) frequently complicates congenital heart disease (CHD). In the current era, life expectancy of patients with PAH-CHD has increased as a consequence of their advanced management in specialized centers and the widespread use of PAH-targeted therapy. Even though, progress has been made in this field, mortality still remains high, primarily due to right heart failure. Aggressive targeting of the underlying pulmonary hypertension and optimal management of fluid balance play a key role in preventing progressive heart failure due to right ventricle dysfunction.

Keywords

Eisenmenger syndrome Endothelin-1 receptor antagonists Phosphodiesterase-5 inhibitors Guanylate cyclase stimulators Prostacyclin receptor agonists 

Abbreviations

ASD

Atrial septal defect

CHD

Congenital heart disease

CMR

Cardiac magnetic resonance

CT

Computed tomography

ES

Eisenmenger syndrome

NT-proBNP

N-terminal pro-B-type natriuretic peptide

NYHA

New York Heart Association

PAH

Pulmonary arterial hypertension

PDA

Patent ductus arteriosus

PH

Pulmonary hypertension

RV

Right ventricle

TAPSE

Tricuspid annular plane systolic excursion

VSD

Ventricular septal defect

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Cardiology Department, AHEPA University HospitalAristotle University of ThessalonikiThessalonikiGreece

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