Abstract 15087: Impact of Early Initiation of Intravenous Prostanoid Therapy in Patients With Congenital Heart Disease Related Pulmonary Hypertension
Background: Congenital heart disease-related pulmonary hypertension (CHD-PH) is associated with considerable morbidity and mortality. While advanced oral therapy (AOT) is now commonly prescribed, intravenous prostanoid therapy (IP) is underutilized due to fears of infection and paradoxical embolism; the impact of this being uncertain.
Methods: We sought to compare the outcomes of patients with CHD-PH initially treated with AOT (endothelin receptor antagonists and phosphodiesterase-5-inhibitors) to those immediately initiated on IP. 51 patients with CHD-PH (40 Eisenmenger’s and 11 corrected shunts) were identified from a prospectively collected database of over 800 patients with PH presenting 1998-2010 to a large tertiary referral center, including 32 patients on AOT and 19 patients on IP.
Results: Patients on AOT and IP were similar in terms of age, sex, body mass index and comorbidities. Baseline 6 minute walk distance (330±138 vs. 292±149 meters) and baseline average WHO function class were also similar (2.7±1.2 of AOT patients and 2.8±1.1 of IP patients), though mean pulmonary artery pressure (50± 23 vs. 68± 19 mmHg, p=0.004) and pulmonary vascular resistance (8.4± 8.2 vs. 15.6± 10.6 Wood units, p=0.016) were higher in IP patients. Use of additional therapies was similar in both groups, though warfarin use trended towards being higher in IP patients (47% vs. 84%, p=0.008). 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).
Conclusions: Early initiation of intravenous prostanoid therapy in CHD-PAH patients appears safe and may significantly prolong their survival.
- © 2013 by American Heart Association, Inc.