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Background: The Fontan palliation causes chronic hepatic congestion and carries a risk of late-adverse cardiac events. VAST and Model for End-stage Liver Disease eXcluding INR (MELD-XI) scores are validated risk-assessment tools in this group. This study investigates whether a combination of these clinical (VAST) and lab-based models (MELD-XI) would improve prediction of adverse cardiac events in Fontan patients.
Methods: Retrospective, two-center study of adult Fontan patients between 2001-16. Adverse events were defined as cardiac death/heart transplantation. VAST score (1 point each Varices, Ascites, Splenomegaly and Thrombocytopenia, range 0-4), MELD-XI score [11.76 (loge creatinine) + 5.112 (loge total bilirubin) + 9.44] and combined M-VAST score (range 0-5), for which a MELD-XI ≥13 was counted as a +1 to VAST score, were compared as predictors of adverse events using receiver operating characteristics (ROC) analysis. Optimal cut-off points were identified using Yoden index. Scores were calculated at a period of outpatient stability. Non-event cohort was censored at last clinical evaluation.
Results: VAST and MELD-XI scores were available in 160 adult patients. After a mean follow-up of 22 yrs, 23 (14%) experienced an adverse event. The event cohort was older (38.7 ±10.3 vs. 31.6 ±8.0 years, p=0.0049), had higher VAST scores (2.2 ±1.2 vs. 0.8 +/-1.0, p<0.0001) and higher MELD-XI scores (12.5 ±3.3 vs. 11.1 ±2.3, p=0.049) compared to non-event cohort. ROC curve for VAST alone as a predictor of adverse events yielded an AUC of 0.80 with the optimal cutoff of ≥2. ROC curve for MELD-XI score yielded an AUC of 0.66 with the optimal cutoff of ≥13. ROC curve for the M-VAST score yielded an improved AUC of 0.82 with the optimal cutoff of M-VAST ≥2. Compared to VAST alone, M-VAST improved sensitivity from 0.70 to 0.78 but decreased specificity from 0.79 to 0.72. Kaplan-Meier analysis with strata divided at M-VAST ≥2 demonstrated improved event free survival of the lower score strata (log rank =0.0002).
Conclusions: Incorporating a MELD-XI into the VAST score, thus combining laboratory and clinical variables, improves the ability to predict death and cardiac transplant in adult Fontan patients.
Poster Hall, Hall C
Saturday, March 18, 2017, 9:45 a.m.-10:30 a.m.
Session Title: Fontan in the Modern World
Abstract Category: 9. Congenital Heart Disease: Adult
Presentation Number: 1183-023
- 2017 American College of Cardiology Foundation