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More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients
Ryan D Byrne1, Angela J Weingarten2, Daniel E Clark2
1Departments of Internal Medicine and Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee.
Insights
Adults with Fontan palliation experience hepatic and renal dysfunction. Systolic ventricular dysfunction and low oxygen saturation are linked to higher MELD and MELD-XI scores, indicating worsening organ function.
Area of Science:
- Cardiology
- Hepatology
- Nephrology
Background:
- Fontan-associated liver disease and chronic kidney disease are common in adults with single ventricle heart disease.
- Assessing extra-cardiac dysfunction is crucial for managing these patients.
Purpose of the Study:
- To evaluate the relationship between Fontan hemodynamics and extra-cardiac dysfunction.
- To determine if invasive and noninvasive measures correlate with MELD and MELD-XI scores.
Main Methods:
- Retrospective analysis of single-center data from adult Fontan patients.
- Invasive hemodynamic data from cardiac catheterization and noninvasive echocardiographic data were collected.
- Linear regression was used to analyze associations with MELD, MELD-XI, and creatinine.
Main Results:
- Decreased systemic oxygen saturation correlated with elevated MELD and MELD-XI scores.
- Reduced ejection fraction was significantly associated with higher MELD and MELD-XI scores.
- Creatinine levels also differed significantly among ventricular dysfunction groups.
Conclusions:
- Systolic ventricular dysfunction is associated with hepatic and renal dysfunction in adults post-Fontan palliation.
- Decreased oxygen saturation is also linked to impaired liver and kidney function.
- MELD and MELD-XI scores serve as indicators of organ dysfunction in this population.
Setting:
Fontan-associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra-cardiac dysfunction as measured by MELD and MELD-XI.
Objective:
We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and MELD-XI scores.
Design:
Single-center data from adults with Fontan palliation who had ongoing care, including cardiac catheterization, were retrospectively collected. Hemodynamic data from cardiac catheterization and echocardiographic assessment of ventricular and atrioventricular valve function were tested for association with serum creatinine, MELD, and MELD-XI. Linear regression was used to perform multivariable analysis in the echocardiogram cohort.
Results:
Fifty-seven patients had congruent lab and catheterization data for analysis. Sixty-three and sixty-nine patients had congruent lab and echocardiogram data for MELD and MELD-XI, respectively. Of the hemodynamic variables analyzed, only decreased systemic oxygen saturation had significant correlation with elevated MELD and MELD-XI (P = .045). Patients with moderately or severely reduced ejection fraction by echocardiogram had significantly higher MELD and MELD-XI scores compared to those with normal or mildly depressed systolic ventricular function (P = .008 and P < .001 for MELD and MELD-XI, respectively). Significant differences in creatinine were also found among the ventricular dysfunction groups (P = .02).
Conclusions:
In adults following Fontan palliation, systolic ventricular dysfunction and decreased oxygen saturation were associated with hepatic and renal dysfunction as assessed by elevated serum creatinine, MELD, and MELD-XI scores.
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