Poor prognosis and related factors in adults with Eisenmenger syndrome

Background Little information is available regarding the clinical history and long-term prognosis of patients with Eisenmenger syndrome without diagnosis until adulthood. The purpose of this study was to investigate the long-term prognosis and factors predictive of the prognosis in patients with Eisenmenger syndrome diagnosis in adulthood. Methods and Results We retrospectively reviewed the clinical data of 106 patients with Eisenmenger syndrome diagnosis in adulthood. Presence of congenital heart defects had neither been noticed nor confirmed with cardiac catheterization until adulthood in most of the patients in our study. After the diagnosis was confirmed, 42 patients died during a mean follow-up period of 7.6 years. The mean survival time from diagnostic catheterization to death was 5.4 years in nonsurvivors. Survival rate was 98% at 1 year, 77% at 5 years, and 58% at 10 years, with the Kaplan-Meier method. Elevated right atrial pressure (>7 mm Hg) and reduced systemic blood flow (<2.9 L/min) were independently associated with increased mortality rate of adults with Eisenmenger syndrome. Conclusion Patients with Eisenmenger syndrome without diagnosis until adulthood had a poor prognosis. Reduced systemic blood flow and elevated right atrial pressure were associated with high mortality rates in adults with Eisenmenger syndrome. (Am Heart J 2002;143:739-44.)