Worsening in oxygen saturation and exercise capacity predict adverse outcome in patients with Eisenmenger syndrome

Abstract Objectives To evaluate (1) changes in clinical, biochemical and echocardiographic parameters, (2) whether deterioration in exercise capacity and resting oxygen saturation (SatO2-rest) are related with adverse outcome and (3) its additional value in predicting outcome in Eisenmenger patients. Methods Seventy-seven (36 ± 14 years, 30% male) patients were included and prospectively followed. Changes between baseline and final visit were evaluated. Clinical deterioration was defined as a deterioration in exercise capacity or SatO2-rest. Univariate and multivariate analyses were performed to evaluate predictors of outcome defined as the need for hospitalization due to right heart failure, transplantation, or all-cause mortality. Finally, the additional prognostic value of deterioration in exercise capacity and SatO2-rest was evaluated. Results During a mean follow-up period of 4.0 ± 2.1 years, 27 (35%) events occurred. Patients in the event-group presented with an deterioration in NYHA class ( P < 0.0001), 6 minute walk distance ( P = 0.006) and SatO2-rest ( P < 0.0001). After adjustment for baseline variables, multivariate Cox regression analysis indicated that clinical deterioration was independently associated with adverse outcome. Conclusions Clinical deterioration, defined as a deterioration in exercise capacity or SatO2-rest was associated with adverse outcome in Eisenmenger patients. Moreover, these parameters provided additional information on which patients would develop an event and may benefit from initiation or escalation of disease targeting therapy.