Dynamic Three-Dimensional Geometry of the Tricuspid Valve Annulus in Hypoplastic Left Heart Syndrome with a Fontan Circulation

• Published in: Journal of the American Society of Echocardiography Vol. 32; no. 5; pp. 655 - 666.e13
• Main Authors: Nguyen, Alex V., Lasso, Andras, Nam, Hannah H., Faerber, Jennifer, Aly, Ahmed H., Pouch, Alison M., Scanlan, Adam B., McGowan, Francis X., Mercer-Rosa, Laura, Cohen, Meryl S., Simpson, John, Fichtinger, Gabor, Jolley, Matthew A.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.05.2019
• Subjects: Adolescent; Adult; Child; Child, Preschool; Congenital heart disease; Echocardiography; Echocardiography, Three-Dimensional; Female; Fontan Procedure; Humans; Hypoplastic Left Heart Syndrome - diagnostic imaging; Hypoplastic Left Heart Syndrome - surgery; Infant; Male; Papillary Muscles - diagnostic imaging; Tricuspid Valve - diagnostic imaging; Tricuspid Valve Insufficiency - diagnostic imaging; Tricuspid Valve Insufficiency - surgery; Valvular heart disease; TV; Echocardiography; A-P; RV; Echo; MS; ICC; Congenital heart disease; TA; ES; HLHS; Valvular heart disease; 3D; 2D; S-L; 3DE; MD; DICOM; APM; TR; ED
• DOI: 10.1016/j.echo.2019.01.002
• ISSN: 0894-7317

Tricuspid regurgitation (TR) is a significant contributor to morbidity and mortality in patients with hypoplastic left heart syndrome. The goal of this study was to characterize the dynamic annular motion of the tricuspid valve in patients with HLHS with a Fontan circulation and assess the relation to tricuspid valve function. Tricuspid annuli of 48 patients with HLHS with a Fontan circulation were modeled at end-diastole, mid-systole, end-systole, and mid-diastole using transthoracic three-dimensional echocardiography and custom code in 3D Slicer. The angle of the anterior papillary muscle (APM) relative to the annular plane in each systolic phase was also measured. Imaging was performed 5.0 years (interquartile range, 2–11 years) after Fontan operation. The tricuspid annulus varies in shape significantly throughout the cardiac cycle, changing in sphericity (P < .001) but not in annular height or bending angle. In univariate modeling, patients with significant TR had larger changes in septolateral diameter, lateral quadrant area, and posterior quadrant area (P < .05 for all) as well as lower (more laterally directed) APM angles (P < .001) than patients with mild or less TR. In multivariate modeling, a 1 mm/(body surface area)0.5 increase in the maximum change in septolateral diameter was associated with a 1.7-fold increase in having moderate or greater TR, while a 10° decrease in APM angle at mid-systole was associated with an almost 2.5-fold increase in moderate or greater TR (P ≤ .01 for all). The tricuspid annulus in patients with HLHS with a Fontan circulation changes in shape significantly throughout the cardiac cycle but remains relatively planar. Increased change in septolateral diameter and decreased APM angle are strongly associated with the presence of TR. These findings may inform annuloplasty methods and subvalvular interventions in these complex patients. •The dynamic TV annuli in 48 patients with HLHS and a Fontan were modeled.•Annular dimensions and shape vary throughout the cardiac cycle.•Change in annular shape and acute papillary muscle angle were associated with TR.