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Updated: Aug 22, 2025
Wireless Telemetry Device Implantation in a Fontan Ovine Model for Continuous and Long-Term Hemodynamic Monitoring
Published on: May 2, 2025
Longitudinal changes in ventricular size and function are associated with death and transplantation late after the
Sunil J Ghelani1,2, Minmin Lu3, Lynn A Sleeper3,4
1Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. sunil.ghelani@cardio.chboston.org.
Insights
Longitudinal ventricular changes in Fontan patients show a decline in function over time. Rapid increases in end-diastolic volume index (EDVI) are linked to adverse outcomes, highlighting the need for close monitoring.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiovascular Imaging
Background:
- Fontan-associated physiology presents unique challenges for ventricular health.
- Previous studies focused on cross-sectional data, leaving longitudinal changes in ventricular function unknown.
Purpose of the Study:
- To investigate longitudinal changes in ventricular structure and function in Fontan patients.
- To correlate these changes with clinical outcomes, including death or heart transplantation.
- To compare remodeling patterns between left ventricular (LV) and right ventricular (RV) dominance.
Main Methods:
- Retrospective analysis of 156 Fontan patients with at least two cardiovascular magnetic resonance (CMR) scans.
- Serial assessment of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), and wall stress.
- Comparison of changes between patients with and without adverse composite outcomes and between LV and RV dominant ventricles.
Main Results:
- Over time, Fontan patient ventricles showed increased volumes (EDVI, ESVI) and wall stress (ESWS), with decreased ejection fraction (EF) and mass.
- A faster increase in EDVI was observed in patients who experienced adverse outcomes (death, transplant).
- Right ventricular (RV) dominance was associated with greater increases in ESVI, decreased EF, and higher rates of adverse outcomes compared to left ventricular (LV) dominance.
Conclusions:
- Fontan circulation is characterized by progressive ventricular decline, evidenced by increasing volumes and wall stress, and decreasing EF.
- Rapidly increasing EDVI may indicate higher risk for adverse events, suggesting closer surveillance.
- Right ventricular dominance portends worse clinical outcomes and adverse ventricular remodeling compared to left ventricular dominance.
Background:
Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known.
Methods:
This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (massi), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance.
Results:
Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2-9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (- 0.61%/year vs. - 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%).
Conclusions:
Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance.
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