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Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease
  1. Peter M Engelfriet1,
  2. Marielle G J Duffels1,
  3. Thomas Möller2,
  4. Eric Boersma3,
  5. Jan G P Tijssen1,
  6. Erik Thaulow4,
  7. Michael A Gatzoulis5,
  8. Barbara J M Mulder1
  1. 1Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands
  2. 2Department of Pediatrics, Vestfold Hospital, Tønsberg, Norway
  3. 3Thoraxcentre, ErasmusMC, Rotterdam, The Netherlands
  4. 4Department of Cardiology, Rikshospitalet, Oslo, Norway
  5. 5Adult Congenital Heart Centre, Royal Brompton Hospital, London, UK
  1. Correspondence to:
    Dr B J M Mulder
    Department of Cardiology, Rm B2-240, Academic Medical Center, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands;b.j.mulder{at}


Aim: To investigate the role of pulmonary arterial hypertension (PAH) in adult patients born with a cardiac septal defect, by assessing its prevalence and its relation with patient characteristics and outcome.

Methods and results: From the database of the Euro Heart Survey on adult congenital heart disease (a retrospective cohort study with a 5-year follow-up), the relevant data on all 1877 patients with an atrial septal defect (ASD), a ventricular septal defect (VSD), or a cyanotic defect were analysed. Most patients (83%) attended a specialised centre. There were 896 patients with an ASD (377 closed, 504 open without and 15 with Eisenmenger’s syndrome), 710 with a VSD (275, 352 and 83, respectively), 133 with Eisenmenger’s syndrome owing to another defect and 138 remaining patients with cyanosis. PAH was present in 531 (28%) patients, or in 34% of patients with an open ASD and 28% of patients with an open VSD, and 12% and 13% of patients with a closed defect, respectively. Mortality was highest in patients with Eisenmenger’s syndrome (20.6%). In case of an open defect, PAH entailed an eightfold increased probability of functional limitations (New York Heart Association class >1), with a further sixfold increase when Eisenmenger’s syndrome was present. Also, in patients with persisting PAH despite defect closure, functional limitations were more common. In patients with ASD, the prevalence of right ventricular dysfunction increased with systolic pulmonary artery pressure (OR = 1.073 per mm Hg; p<0.001). Major bleeding events were more prevalent in patients with cyanosis with than without Eisenmenger’s syndrome (17% vs 3%; p<0.001).

Conclusion: In this selected population of adults with congenital heart disease, PAH was common and predisposed to more symptoms and further clinical deterioration, even among patients with previous defect closure and patients who had not developed Eisenmenger’s physiology.

  • ASD, atrial septal defect
  • NYHA, New York Heart Association
  • PAH, pulmonary arterial hypertension
  • sPAP, systolic pulmonary artery pressure
  • VSD, ventricular septal defect

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  • Published Online First 5 December 2006

  • Competing interests: None declared.