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.