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Pulmonary arterial hypertension in adults born with a heart septal defect
  1. Peter M Engelfriet (p.m.engelfriet{at}
  1. Academic Medical Centre, Amsterdam, Netherlands
    1. Marielle G J Duffels (m.g.duffels{at}
    1. Academic Medical Centre, Amsterdam, Netherlands
      1. Thomas Moeller (f-moel{at}
      1. Vestfold Hospital, Toensberg, Norway
        1. Eric Boersma (h.boersma{at}
        1. Erasmus Medical Center, Netherlands
          1. Jan G P Tijssen (j.tijssen{at}
          1. Academic Medical Centre, Amsterdam, Netherlands
            1. Erik Thaulow (erik.thaulow{at}
            1. Rikshospitalet, Norway
              1. Michael A Gatzoulis (m.gatzoulis{at}
              1. Royal Brompton Hospital, London, United Kingdom
                1. Barbara J M Mulder (b.j.mulder{at}
                1. Academic Medical Centre, Amsterdam, Netherlands


                  Aims 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 syndrome), 710 with a VSD (275, 352, 83, respectively), 133 with Eisenmenger due to another defect, and 138 remaining cyanotic patients. PAH was present in 531 patients (28%), or in 34% of patients with an open ASD and 28% of patients with an open VSD, and 12%, respectively 13%, of patients with a closed defect. Mortality was highest in Eisenmenger patients: 20.6%. In case of an open defect, PAH entailed an eight-fold increased probability of functional limitations (NYHA class > 1), with a further six-fold increase when Eisenmenger syndrome was present. Also in patients with persisting PAH despite defect closure, functional limitations were more common. In ASD patients, the prevalence of right ventricular dysfunction increased with systolic pulmonary artery pressure (odds ratio 1.073 per mm Hg; P < 0.001). Major bleeding events were more prevalent in cyanotic patients with than without Eisenmenger syndrome (17% versus 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 amongst patients with previous defect closure and patients who had not developed Eisenmenger physiology.

                  • Eisenmenger syndrome
                  • Euro Heart Survey
                  • Pulmonary arterial hypertension
                  • pulmonary artery pressure
                  • septal defect

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