Pulmonary vasodilator therapy is associated with greater survival in Eisenmenger syndrome

Clare Arnott; Geoff Strange; Andrew Bullock; Adrienne C Kirby; Clare O'Donnell; Dorothy J Radford; Leeanne E Grigg; David S Celermajer

doi:10.1136/heartjnl-2017-311876

Pulmonary vasodilator therapy is associated with greater survival in Eisenmenger syndrome

Heart. 2017 Aug 9:heartjnl-2017-311876. doi: 10.1136/heartjnl-2017-311876. Online ahead of print.

Authors

Clare Arnott^{1

2}, Geoff Strange^{3

4}, Andrew Bullock^{5

6}, Adrienne C Kirby⁷, Clare O'Donnell^{8

9}, Dorothy J Radford^{10

11}, Leeanne E Grigg^{12

13}, David S Celermajer^{1

2}

Affiliations

¹ Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
² Sydney Medical School, University of Sydney - Camperdown and Darlington Campus, Sydney, New South Wales, Australia.
³ Pulmonary Hypertension Society ANZ, Sans Souci, New South Wales, Australia.
⁴ Faculty of Medicine, University of Notre Dame, Sydney, New South Wales, Australia.
⁵ Department of Cardiology, Royal Perth Hospital, Perth, Western Australia, Australia.
⁶ Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, Western Australia, Australia.
⁷ National Health and Medical Research Council Clinical Trials Centre, Biostatistics, Camperdown, New South Wales, Australia.
⁸ Department of Paediatric Cardiology, Auckland City Hospital, Auckland, New Zealand.
⁹ School of Medicine, University of Auckland, Auckland, New Zealand.
¹⁰ Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Queensland, Australia.
¹¹ Department of Medicine, University of Queensland, Brisbane, Queensland, Australia.
¹² Faculty of Medicine, University of Melbourne, Melbourne, Victoria, Australia.
¹³ Department of Cardiology, Royal Melbourne Hospital, Melbourne, Victoria, Australia.

PMID: 28794135
DOI: 10.1136/heartjnl-2017-311876

Abstract

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.

Keywords: Complex Congenital Heart Disease; Congenital Heart Disease; Pulmonary Vascular Disease.