Key result
Moderate or greater tricuspid regurgitation in hypoplastic left heart syndrome with a Fontan circulation was associated with greater leaflet billow volume and altered papillary muscle angle (P<0.001).
Why the study?
In hypoplastic left heart syndrome, tricuspid regurgitation is associated with circulatory failure and death, but whether tricuspid valve structure and right ventricle volume differ by regurgitation severity was unproven.
Cross-Sectional (n=100)
Effect estimate: C statistic 0.85
p-value: p=<0.001
In patients with hypoplastic left heart syndrome and a Fontan circulation, moderate or greater tricuspid regurgitation is associated with specific structural features and significant heterogeneity, suggesting a need for image-informed patient-specific surgical planning.
May support image-informed planning in HLHS Fontan; leaves open whether modifying these features alters TR outcomes.
Background: In hypoplastic left heart syndrome, tricuspid regurgitation (TR) is associated with circulatory failure and death. We hypothesized that the tricuspid valve (TV) structure of patients with hypoplastic left heart syndrome with a Fontan circulation and moderate or greater TR differs from those with mild or less TR, and that right ventricle volume is associated with TV structure and dysfunction. Methods: TV of 100 patients with hypoplastic left heart syndrome and a Fontan circulation were modeled using transthoracic 3-dimensional echocardiograms and custom software in SlicerHeart. Associations of TV structure to TR grade and right ventricle function and volume were investigated. Shape parameterization and analysis was used to calculate the mean shape of the TV leaflets, their principal modes of variation, and to characterize associations of TV leaflet shape to TR. Results: In univariate modeling, patients with moderate or greater TR had larger TV annular diameters and area, greater annular distance between the anteroseptal commissure and anteroposterior commissure, greater leaflet billow volume, and more laterally directed anterior papillary muscle angles compared to valves with mild or less TR (all P <0.001). In multivariate modeling greater total billow volume, lower anterior papillary muscle angle, and greater distance between the anteroposterior commissure and anteroseptal commissure were associated with moderate or greater TR ( P <0.001, C statistic=0.85). Larger right ventricle volumes were associated with moderate or greater TR ( P <0.001). TV shape analysis revealed structural features associated with TR, but also highly heterogeneous TV leaflet structure. Conclusions: Moderate or greater TR in patients with hypoplastic left heart syndrome with a Fontan circulation is associated with greater leaflet billow volume, a more laterally directed anterior papillary muscle angle, and greater annular distance between the anteroseptal commissure and anteroposterior commissure. However, there is significant heterogeneity of structure in the TV leaflets in regurgitant valves. Given this variability, an image-informed patient-specific approach to surgical planning may be needed to achieve optimal outcomes in this vulnerable and challenging population.
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Nam et al. (2023) conducted a cross-sectional in Hypoplastic left heart syndrome with a Fontan circulation (n=100). Moderate or greater tricuspid regurgitation vs. Mild or less tricuspid regurgitation was evaluated on Tricuspid valve structural differences (e.g., greater leaflet billow volume, lower anterior papillary muscle angle) (C statistic 0.85, p=<0.001). Moderate or greater tricuspid regurgitation in hypoplastic left heart syndrome with a Fontan circulation was associated with greater leaflet billow volume and altered papillary muscle angle (P<0.001).
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