Abstract 18725: Current Era Survival and Prevalence of Pulmonary Hypertension-Specific Therapy in Adult Patients With Eisenmenger Syndrome
Background: We have previously reported that patients with Eisenmenger syndrome (ES) without diagnosis until adulthood had a poor prognosis. Those patients were enrolled before 1998, when any pulmonary hypertension (PH)-specific therapies were unavailable in Japan. Thereafter, several PH-specific therapies have been markedly developing. However, it remains to be elucidated whether or not increasing use of modern PH treatment leads to improved prognosis in ES patients in the current era, compared with the previous era. This study aimed to investigate (1) the prevalence of modern PH treatment and (2) long-term prognosis in adult patients with ES in the current era.
Methods: We retrospectively studied 38 consecutive adult patients (42±16 years; 25 women) with ES who underwent diagnostic catheterization between January 2001 and January 2016 in our center. The prevalence of modern PH treatment; a long-acting oral prostacyclin analogue, an endothelin receptor antagonist, a phosphodiesterase type-5 inhibitor, and intravenous epoprostenol and its impact on hemodynamics and long-term prognosis were studied and compared with our previous report including 106 adult patients with ES who were treated with supportive therapy alone (historical control group).
Results: Of 38 patients, 33 (87%) were treated with modern PH treatment (modern group), including oral triple combination therapy in 10, while 5 were treated without modern PH treatment throughout the follow-up (non-modern group). No significant differences were found for any baseline characteristics between modern and non-modern groups. During a mean follow-up of 7.3±3.9 years, 7 patients died and 3 underwent lung transplantation. Kaplan-Meier analysis showed that transplantation free-survival in modern group was 100% at 1 year, 90% at 5 years, and 81% at 10 years, which was significantly higher than non-modern group (75% at 1 year, 50% at 5 years, and 25% at 10 years) (P<0.05) and eventually historical control group (98% at 1 year, 77% at 5 years, and 58% at 10 years).
Conclusions: Adult patients with ES treated with modern PH treatment had better long-term prognosis than those in non-modern and historical control groups, which may be associated with proactive use of modern PH treatment over the last decade.
Author Disclosures: H. Matama: None. S. Fukui: None. T. Ogo: None. J. Ueda: None. A. Tsuji: None. T. Noguchi: None. K. Kusano: None. T. Anzai: None. S. Yasuda: None.
- © 2016 by American Heart Association, Inc.