Abstract 1792: Improved Survival Amongst Patients With Eisenmenger Syndrome Receiving Advanced Therapy for Pulmonary Arterial Hypertension
Background: Advanced therapy (AT) for pulmonary arterial hypertension in the context of congenital heart disease (Eisenmenger syndrome) improves pulmonary hemodynamics, functional class and the 6-minute walk test. We examined the potential effect of AT on survival in this population.
Methods and Results: Data on all Eisenmenger patients attending our centre over the last decade were collected. Survival was compared between patients on and off AT by Cox regression analysis, adjusting for baseline differences by propensity scores. Patients initiated on AT during follow-up were reassessed and treated as separate cases (right-censored and left-truncated). A total of 229 patients (age 35.5±12.5, 35.4% male) were included. The majority had complex anatomy and 61.7% were in NYHA III or more at baseline assessment. Mean resting saturations were 84.0%. Sixty eight (29.7%) patients were on AT, or AT was initiated during follow-up. Among those who received AT, 73.5% were started on bosentan, 25% on sildenafil. During a median follow-up of 3.7 years, 52 patients died, only 2 of them while on AT. Patients on AT were at a significantly lower risk of death, both unadjusted and after adjustment for baseline clinical differences by propensity score regression adjustment (C-statistic=0.79, HR 0.16, 95% CI: 0.04 – 0.69, p=0.01) and propensity score matching (HR 0.18, 95% CI: 0.04 – 0.79, p=0.02).
Conclusions: AT for pulmonary arterial hypertension in a contemporary cohort of adults with Eisenmenger syndrome was associated with a lower risk of death. Survival benefits should be considered together with improved hemodynamics and functional class when decisions are made on AT in this population.