Abstract 15098: Impact of Early Initiation of Intravenous Prostanoid Therapy in Patients With Eisenmenger Syndrome
Background: Eisenmenger Syndrome (ES) is the most advanced form of shunt-related pulmonary vascular disease and associated with considerable morbidity and mortality. While advanced oral therapy is now commonly prescribed in ES, intravenous therapy is underutilized due to fears of infection and paradoxical embolism; the impact of this choice is uncertain.
Methods: We sought to compare the outcomes of patients with ES initially treated with advanced oral therapy (AOT; endothelin receptor antagonists and phosphodiesterase-5-inhibitors) to those immediately initiated on intravenous prostanoids (IP). We identified 40 patients with ES from a prospectively collected database of over 800 patients with pulmonary hypertension presenting between 1998-2010 to a large tertiary referral center, including 27 patients on AOT and 13 patients on IP. All patients had unrepaired shunts and were desaturated at rest or with exertion. Survival was compared using Kaplan-Meier analysis.
Results: Patients on AOT and IP were similar in terms of age, sex, body mass index and comorbidities. Baseline 6 minute walk distance (329±142 vs. 302±146 meters) and WHO function class were also similar (III or IV in 52% of AOT patients and 46% of IP patients), though mean pulmonary artery pressure (50±25 vs. 71±18 mmHg, p=0.004) and pulmonary vascular resistance (7.8±8.6 vs. 18.4±10.3 Wood units, p=0.004) were higher in the IP patients. Use of additional therapies was similar in both groups, though warfarin use trended towards being higher in IP patients (44% vs. 77%, p=0.053). There were no paradoxical embolic events or life-threatening infections in either group, though survival appeared markedly improved in ES patients treated upfront with IP (Figure). In 6 patients receiving AOT, IP was eventually added, though 5 of these patients subsequently died.
Conclusion: Early initiation of intravenous prostanoid therapy in Eisenmenger patients appears safe and may significantly prolong their survival.
- © 2013 by American Heart Association, Inc.