Abstract 8732: Predictors of Mortality and the Effect of Disease Specific Therapy on Survival in Adult Patients With Eisenmenger Syndrome
Background: Disease specific therapy (DST) is expected to improve survival of adult patients with Eisenmenger syndrome (ES). However, there are few articles on survival in current era of DST. Our aim is to assess survival rate and predictors of mortality in ES patients and clarify the effect of DST on survival in these patients.
Methods: Clinical course and data were collected and analyzed in 199 patients (male 72, a median age: 35 yrs, range: 16–;69 yrs) with ES who visited outpatient clinic of 16 participating institutes in Japan and Korea from 1998 to 2009. Clinical data at first clinic visits during adulthood and last clinic visits were examined. Kaplan-Meier survival curve was constructed and survival rates among groups were compared by log-rank test. Predictive factors of mortality were determined by multivariate stepwise regression analysis.
Results: During a median follow-up of 14.9 yrs, thirty patients died and fourteen of them died suddenly. Survival rate at age 40, 50, 60yrs was 86, 74, 53% respectively. There was no significant difference in mortality between simple and complex anatomies. 40 years survival was lower in patients with a history of syncope than those without (60% vs 87%, p=0.009), despite a history of arrhythmia was not a predictor for mortality. In a univariate analysis of data at first visit, platelet cell count<14.7×104/mm3, Uric acid >7.7mg/dl, BNP >124pg/ml were associated with mortality, and only BNP>124pg/ml was an independent predictor (Risk ratio(RR) 3.2, p=0.005). Moreover, in a multivariate analysis of data at last visit, SpO2<82% (RR 4.3, p=0.006), Body weight<47kg (RR 2.6, p=0.047), BNP>180pg/ml (RR 21, p<0.0001) were related to mortality. In eighty six patients with NYHA functional class≥III at last clinic visits, survival rate in patients taking DST (n=48) was significantly higher than those without (78% vs 58% at 10 years, log rank test; p=0.01).
Conclusions: DST can improve prognosis of patients with ES. So, the predictive factors such as a history of syncope, thrombocytepeny and high serum level of BNP and uric acid are helpful to make a decision on initiation of DST. Finally, meticulous care for prevention of severe hypoxemia and low body weight and high serum level of BNP may be helpful for better prognosis.
- © 2010 by American Heart Association, Inc.