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Original research article
Pulmonary vasodilator therapy is associated with greater survival in Eisenmenger syndrome
  1. Clare Arnott1,2,
  2. Geoff Strange3,4,
  3. Andrew Bullock5,6,
  4. Adrienne C Kirby7,
  5. Clare O’Donnell8,9,
  6. Dorothy J Radford10,11,
  7. Leeanne E Grigg12,13,
  8. David S Celermajer1,2
  1. 1 Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
  2. 2 Sydney Medical School, University of Sydney - Camperdown and Darlington Campus, Sydney, New South Wales, Australia
  3. 3 Pulmonary Hypertension Society ANZ, Sans Souci, New South Wales, Australia
  4. 4 Faculty of Medicine, University of Notre Dame, Sydney, New South Wales, Australia
  5. 5 Department of Cardiology, Royal Perth Hospital, Perth, Western Australia, Australia
  6. 6 Children’s Cardiac Centre, Princess Margaret Hospital for Children, Perth, Western Australia, Australia
  7. 7 National Health and Medical Research Council Clinical Trials Centre, Biostatistics, Camperdown, New South Wales, Australia
  8. 8 Department of Paediatric Cardiology, Auckland City Hospital, Auckland, New Zealand
  9. 9 School of Medicine, University of Auckland, Auckland, New Zealand
  10. 10 Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Queensland, Australia
  11. 11 Department of Medicine, University of Queensland, Brisbane, Queensland, Australia
  12. 12 Faculty of Medicine, University of Melbourne, Melbourne, Victoria, Australia
  13. 13 Department of Cardiology, Royal Melbourne Hospital, Melbourne, Victoria, Australia
  1. Correspondence to Dr Clare Arnott, Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, 2050, NSW, Australia; carn8379{at}


Objective Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT).

Methods From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand. Demographic, medical and outcome data were collected and analysed prospectively and retrospectively.

Results The patients with ES were predominantly female (60%), aged 31 (SD 12) years. At diagnosis of ES, 64% were WHO functional class ≥3. The most common underlying lesion was ventricular septal defect (33%) with 21% having ‘complex’ anatomy. Over a median follow-up time of 9.1 years, the majority (72%) had been prescribed at least one AT (49% single agent), mostly bosentan (66%, 168 patients). The mean time on AT was 6 (SD 3.6) years. Those on AT were more functionally impaired at presentation (69% WHO ≥3 vs 51%, p=0.007) and more likely to have been prescribed anticoagulation (47% vs 27%, p=0.003). The risk of death/transplant was 4.8 %/year in AT exposed versus 8.4% in those never exposed. On multivariable analysis, exposure to AT was independently associated with greater survival (survival HR 2.27, 95% CI 1.49 to 3.45; p<0.001). WHO ≥3 at presentation was associated with a worse prognosis (mortality HR 1.82, 95% CI 1.19 to 2.78; p=0.006).

Conclusion Treatment with AT was independently associated with greater survival in patients with ES, even though they were comparatively sicker prior to treatment.

  • Congenital Heart Disease
  • Pulmonary Vascular Disease
  • Complex Congenital Heart Disease

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  • Contributors All authors are responsible for the reported research and have approved the manuscript for submission. Specifically, CA and DSC were responsible for initial study concept, primary data collection and analysis and manuscript preparation and submission. They are responsible for overall content as guarantors. GS assisted with concept design, data collection and manuscript preparation. AB, CO, DJR and LG assisted with data collection, manuscript preparation and correction. ACK assisted with data analysis, manuscript preparation and correction.

  • Competing interests None declared.

  • Ethics approval Local ethics approval was obtained at each contributing site across Australia and New Zealand.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement There is no additional unpublished data from this study available to other parties.

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