title: Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification.
abstract: BACKGROUND
  The aging congenital heart disease (CHD) population is prone to develop a variety of sequelae, including pulmonary arterial hypertension (PAH). Previous prevalence estimates are limited in applicability due to the use of tertiary centers, or database encoding only. We aimed to investigate the contemporary prevalence of PAH in adult CHD patients, using a nationwide population.
  
  
  METHODS
  A cross-sectional study was performed, using the population-based Dutch CONgenital CORvitia (CONCOR) registry. All patients born with a systemic-to-pulmonary shunt, thereby at risk of developing PAH, were identified. From this cohort, a random sample was obtained and carefully reviewed.
  
  
  RESULTS
  Of 12,624 registered adults with CHD alive in 2011, 5,487 (44%) were at risk of PAH. The random sample consisted of 1,814 patients (mean age 40 ± 15 years) and 135 PAH cases were observed. PAH prevalence in patients born with a systemic-to-pulmonary shunt was 7.4%. The prevalence of PAH after corrective cardiac surgery was remarkably high (5.7%). Furthermore, PAH prevalence increased with age, from 2.5% under 30 years until 35% in the eldest. PAH prevalence in the entire CHD population was 3.2%. Based on 3000 per million adult CHD patients in the general population, we can assume that PAH-CHD is present in 100 per million.
  
  
  CONCLUSIONS
  This new approach using a nationwide CHD population reports a PAH prevalence of 3.2% in CHD patients, and 100 per million in the general adult population. Especially in patients after shunt closure and the elderly, physicians should be aware of PAH-CHD, to provide optimal therapeutic and clinical care.
authors.affiliation: Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands.
authors.forename: Annelieke C M J
authors.initials: AC
authors.lastname: van Riel
authors.affiliation: Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands.
authors.forename: Mark J
authors.initials: MJ
authors.lastname: Schuuring
authors.affiliation: Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands.
authors.forename: Irene D
authors.initials: ID
authors.lastname: van Hessen
authors.affiliation: Department of Clinical Epidemiology and Biostatistics, Academic Medical Centre, Amsterdam.
authors.forename: Aielko H
authors.initials: AH
authors.lastname: Zwinderman
authors.affiliation: Department of Cardiology, Gelre Hospital, Apeldoorn, The Netherlands.
authors.forename: Luc
authors.initials: L
authors.lastname: Cozijnsen
authors.affiliation: Department of Cardiology, Medical Centre Alkmaar, Alkmaar, The Netherlands.
authors.forename: Constant L A
authors.initials: CL
authors.lastname: Reichert
authors.affiliation: Department of Cardiology, Isala Klinieken, Zwolle, The Netherlands.
authors.forename: Jan C A
authors.initials: JC
authors.lastname: Hoorntje
authors.affiliation: Department of Cardiology, Medisch Spectrum Twente Hospital, Enschede, The Netherlands.
authors.forename: Lodewijk J
authors.initials: LJ
authors.lastname: Wagenaar
authors.affiliation: Department of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands.
authors.forename: Marco C
authors.initials: MC
authors.lastname: Post
authors.affiliation: Department of Cardiology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Arie P J
authors.initials: AP
authors.lastname: van Dijk
authors.affiliation: Department of Cardiology, University Medical Centre Groningen, Groningen, The Netherlands.
authors.forename: Elke S
authors.initials: ES
authors.lastname: Hoendermis
authors.affiliation: Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands.
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
authors.affiliation: Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands. Electronic address: b.j.bouma@amc.nl.
authors.forename: Berto J
authors.initials: BJ
authors.lastname: Bouma
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000150:complications* / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D015995:Prevalence
keywords: Adult congenital heart disease; Echocardiography; Epidemiology; Prevalence; Pulmonary arterial hypertension