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Longitudinal study of anthropometry in Fontan survivors: Pediatric Heart Network Fontan study
Linda M Lambert1, Brian W McCrindle2, Victoria L Pemberton3
1University of Utah/Primary Children's Hospital, Salt Lake City, UT.
Insights
Fontan survivors often have shorter stature compared to the general population, which is linked to poorer survival rates. Improved height and BMI over time correlate with better quality of life and exercise capacity.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Growth and Development
Background:
- Single-ventricle survivors face growth abnormalities impacting quality of life (QoL) and exercise capacity.
- The Fontan procedure, a palliative surgery for complex congenital heart defects, leads to unique long-term physiological challenges.
Purpose of the Study:
- To evaluate longitudinal changes in height and body mass index (BMI) in Fontan survivors.
- To determine the relationship between growth parameters, mortality, ventricular morphology, QoL, and exercise capacity.
Main Methods:
- Multicenter, longitudinal analysis of participants in the Pediatric Heart Network Fontan studies.
- Comparison of height and BMI z-scores to population norms across three time points (F1, F2, F3).
- Statistical analysis of associations between growth parameters and clinical outcomes.
Main Results:
- Fontan survivors exhibited lower height z-scores compared to population norms across all study phases.
- Shorter stature was significantly associated with increased mortality.
- Dominant right ventricular morphology was linked to lower height z-scores.
- Increased height z-score correlated with improved QoL and exercise capacity (oxygen consumption).
- In participants over 20 years old, increasing BMI was associated with decreased exercise capacity.
Conclusions:
- Fontan survivors, particularly those with right ventricular morphology, are shorter than the general population, with similar BMI.
- Shorter stature is a predictor of worse survival outcomes.
- Improvements in height and BMI over time are associated with enhanced QoL and exercise capacity, though increased BMI in older adults may negatively impact exercise performance.
Background:
Growth abnormalities in single-ventricle survivors may reduce quality of life (QoL) and exercise capacity.
Methods:
This multicenter, longitudinal analysis evaluated changes in height and body mass index (BMI) compared to population norms and their relationship to mortality, ventricular morphology, QoL, and exercise capacity in the Pediatric Heart Network Fontan studies.
Results:
Fontan 1 (F1) included 546 participants (12 ± 3.4 years); Fontan 2 (F2), 427 (19 ± 3.4 years); and Fontan 3 (F3), 362 (21 ± 3.5 years), with ~60% male at each time point. Height z-score was -0.67 ± -1.27, -0.60 ± 1.34, and- 0.43 ± 1.14 at F1-F3, lower compared to norms at all time points (P ≤ .001). BMI z-score was similar to population norms. Compared to survivors, participants who died had lower height z-score (P ≤ .001). Participants with dominant right ventricle (n = 112) had lower height z-score (P ≤ .004) compared to dominant left (n = 186) or mixed (n = 64) ventricular morphologies. Higher height z-score was associated with higher Pediatric Quality of Life Inventory for the total score (slope = 2.82 ± 0.52; P ≤ .001). Increase in height z-score (F1 to F3) was associated with increased oxygen consumption (slope = 2.61 ± 1.08; P = .02), whereas, for participants >20 years old, an increase in BMI (F1 to F3) was associated with a decrease in oxygen consumption (slope = -1.25 ± 0.33; P ≤ .001).
Conclusions:
Fontan survivors, especially those with right ventricular morphology, are shorter when compared to the normal population but have similar BMI. Shorter stature was associated with worse survival. An increase in height z-score over the course of the study was associated with better QoL and exercise capacity; an increase in BMI was associated with worse exercise capacity.
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