Pulmonary Arterial Hypertension

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


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.


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



Atrial septal defect


Congenital heart disease


Cardiac magnetic resonance


Computed tomography


Eisenmenger syndrome


N-terminal pro-B-type natriuretic peptide


New York Heart Association


Pulmonary arterial hypertension


Patent ductus arteriosus


Pulmonary hypertension


Right ventricle


Tricuspid annular plane systolic excursion


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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