Prognostic Role of Hepatorenal Function Indexes in Patients With Ebstein Anomaly

Abstract

Background: Hepatorenal dysfunction is a risk factor for mortality in patients with chronic tricuspid regurgitation due to acquired heart disease. Ebstein anomaly is the most common cause of primary tricuspid regurgitation in adults with congenital heart disease, but the prevalence and prognostic implications of hepatorenal dysfunction are unknown in this population.

Objectives: The purpose of this study was to determine the risk factors and prognostic implications of hepatorenal dysfunction, as measured primarily by the use of model for end-stage liver disease excluding international normalized ratio (MELD-XI score), as well as looking at other associated factors.

Methods: This was a retrospective study of adults with Ebstein anomaly who received care at Mayo Clinic from 2003 to 2018.

Results: Of 692 patients, the median MELD-XI score was 10.2 (interquartile range: 9.4 to 13.3); 53 (8%) died and 3 (0.4%) underwent heart transplant. MELD-XI was an independent predictor of death/transplant (hazard ratio: 1.32; 95% confidence interval: 1.11 to 2.06; p < 0.001). In the subset of patients with serial MELD-XI scores (n = 416), temporal change in MELD-XI score (ΔMELD-XI) was also a predictor of death/transplant. In the subset of patients who underwent tricuspid valve surgery (n = 344), a post-operative improvement in MELD-XI score (ΔMELD-XI) was associated with improved long-term survival. Impaired right atrial (RA) reservoir strain and elevated estimated RA pressure were associated with worse baseline MELD-XI and ΔMELD-XI scores.

Conclusions: Hepatorenal dysfunction is a predictor of mortality in Ebstein anomaly, and RA dysfunction and hypertension are hemodynamic biomarkers that can identify patients at risk for deterioration in hepatorenal function and mortality. These data highlight the prognostic importance of noncardiac organ-system dysfunction, and provide complementary clinical risk stratification metrics for management of these patients.

Keywords: Ebstein anomaly; hepatorenal dysfunction; mortality.

Figure 1:

Flowchart and Kaplan Meier curves showing the impact of postoperative change in hepatorenal function on survival. Top: Flowchart showing patients that had high MELD-XI score at preoperative and postoperative assessments (red), patients that postoperative improvement in MELD-XI score, i.e. moved from high to low MELD-XI score after surgery (blue), and patients that had low MELD-XI score at preoperative and postoperative assessments (black). Bottom: Compared to the patients that remained in the high MELD-XI class (red), those that had postoperative reduction in MELD-XI score (blue) had better 10-year transplant-free survival (71% vs 86%, p=0.001). However 10-year transplant-free survival was similar between the patients with postoperative improvement in MELD-XI score (blue) and those that remained in the low MELD-XI group (black) (86% vs 84%, p=0.7). p=0.04 represent curve comparison across all 3 groups using log-rank test.

Central illustration:. Flowchart and Kaplan Meier curves showing the impact of deterioration in hepatorenal function on survival.

Top: Flowchart showing patients that had low MELD-XI score at baseline and during follow-up (black), patients that moved from low to high MELD-XI score during follow-up (blue), and patients that had high MELD-XI score at baseline and during follow-up (red). Bottom: Compared to the patients that remained in the low MELD-XI class (black), those that developed high MELD-XI score during follow-up (blue) had lower 210-year transplant-free survival (97% vs 86%, p=0.006). Patients with high MELD-XI score at baseline and during follow-up had the lowest survival of the 3 groups (78%). p=0.01 represent curve comparison across all 3 groups using log-rank test.