Fontan Route Remodeling over Time: A Longitudinal Quantitative 3D Case Series

Fontan patients experience anatomical remodeling over time, yet the mechanisms driving these changes remain unclear. This study aimed to characterize full-route Fontan remodeling and evaluate whether observed morphological changes arise from somatic growth alone or from the combined influence of conduit properties, surgical design, thoracic anatomy, and mechanical forces. Five Fontan patients (four extracardiac, one lateral tunnel) underwent analysis using two MRI-derived 3D models obtained between 1 and 4 years apart. Directional displacement was assessed using 3D shape overlays, surface geometry was quantified using the Koenderink Shape Index (KSI), and patient-specific growth mapping estimated localized tissue dynamics. Statistical analyses included a one-sample <i>t</i>-test for mean anterior displacement, the Grubbs' test for outlier detection, and the Wilcoxon signed-rank test for KSI comparisons across time points. All patients exhibited anterior displacement of the Fontan route, with a mean shift of 0.29″ ± 0.33″ and one significant outlier (lateral tunnel, 0.87″). Four of five patients showed increased convexity over time. Growth mapping revealed minimal, heterogeneous native-tissue expansion, with localized growth up to 0.2 mm/year. Individual remodeling trajectories varied and did not consistently align with localized anterior growth, indicating that Fontan route remodeling is highly individualized and cannot be explained by somatic growth alone. This retrospective longitudinal case series study highlights the value of quantitative 3D geometric tools for assessing subtle Fontan route remodeling and supports the feasibility of growth-aware, patient-specific modeling frameworks in single-ventricle physiology.

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Publication Details

Journal
Journal of Cardiovascular Development and Disease
Published
2026-01-13
DOI
https://doi.org/10.3390/jcdd13010045
Primary Topic
Congenital Heart Disease Studies
Type
article
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article

Fontan Route Remodeling over Time: A Longitudinal Quantitative 3D Case Series

Narutoshi Hibino, Amartya Dave, N. Nguyen, Luka Pocivavsek et al.
Journal of Cardiovascular Development and Disease
Congenital Heart Disease Studies
article

Fontan Route Remodeling over Time: A Longitudinal Quantitative 3D Case Series

Narutoshi Hibino, Amartya Dave, N. Nguyen, Luka Pocivavsek, Raquel dos Santos, Kameel Khabaz, Aashi Dharia, Deqa Muse, Junsung Kim, Mohammed Usmaan Siddiqi
article en

Abstract

Fontan patients experience anatomical remodeling over time, yet the mechanisms driving these changes remain unclear. This study aimed to characterize full-route Fontan remodeling and evaluate whether observed morphological changes arise from somatic growth alone or from the combined influence of conduit properties, surgical design, thoracic anatomy, and mechanical forces. Five Fontan patients (four extracardiac, one lateral tunnel) underwent analysis using two MRI-derived 3D models obtained between 1 and 4 years apart. Directional displacement was assessed using 3D shape overlays, surface geometry was quantified using the Koenderink Shape Index (KSI), and patient-specific growth mapping estimated localized tissue dynamics. Statistical analyses included a one-sample <i>t</i>-test for mean anterior displacement, the Grubbs' test for outlier detection, and the Wilcoxon signed-rank test for KSI comparisons across time points. All patients exhibited anterior displacement of the Fontan route, with a mean shift of 0.29″ ± 0.33″ and one significant outlier (lateral tunnel, 0.87″). Four of five patients showed increased convexity over time. Growth mapping revealed minimal, heterogeneous native-tissue expansion, with localized growth up to 0.2 mm/year. Individual remodeling trajectories varied and did not consistently align with localized anterior growth, indicating that Fontan route remodeling is highly individualized and cannot be explained by somatic growth alone. This retrospective longitudinal case series study highlights the value of quantitative 3D geometric tools for assessing subtle Fontan route remodeling and supports the feasibility of growth-aware, patient-specific modeling frameworks in single-ventricle physiology.

Journal of Cardiovascular Development and DiseaseVol. 13(1)
University of California, Los Angeles (US), University of California, San Francisco (US), Yale University (US), University of Chicago (US)
Sustainable cities and communities
Openalex Percentile: Top 89%
Congenital Heart Disease Studies
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