Cardiovascular

Longitudinal Remodeling and Network Architecture of the Proximal Aorta in Children and Adolescents With Marfan Syndrome.

TL;DR

In 515 children and adolescents with Marfan syndrome from the Pediatric Heart Network trial, all proximal aortic absolute diameters increased over 36 months while Z-scores decreased, and the between-patient network of aortic segments was stronger than the within-patient network, so network findings complement but do not replace serial measurement of individual aortic levels.

Key Findings

The analysis included 515 participants from the Pediatric Heart Network Marfan trial, most of whom had complete echocardiographic follow-up.

  • Secondary analysis of Pediatric Heart Network Marfan trial data.
  • Participants were aged <18 years at randomization and had acceptable echocardiograms at baseline and at 6, 12, 24, and 36 months.
  • 445 participants (86.4%) had five acceptable visits.

From baseline to 36 months, absolute diameters of all analyzed proximal aortic segments increased.

  • Segments analyzed were the aortic annulus, sinuses of Valsalva, sinotubular junction, and ascending aorta.
  • Increases in absolute diameter were seen across all segments.

Z-scores of the proximal aortic segments decreased over 36 months despite increases in absolute diameter.

  • Z-scores decreased from -0.688 to -0.454 (all p < 0.001), as reported in the abstract.
  • This indicates that aortic growth was slower than expected for body size growth over the period.

Exploratory age-stratified analyses showed larger Z-score decreases among participants aged 6-11 years.

  • The age-stratified analyses were described as exploratory.
  • Larger decreases were seen in the 6-11 year group compared with other age strata.

The interindividual network of the proximal aorta was stronger than the intraindividual network.

  • Global strength was 1.783 for the interindividual network vs 1.020 for the intraindividual network.
  • Difference was 0.763 (95% CI 0.526-1.101; p < 0.001).
  • Partial correlation networks separated interindividual and intraindividual components; uncertainty was assessed with participant-level bootstrap resampling.

Sensitivity analyses showed the same overall pattern of declining Z-scores.

  • Sensitivity analyses used inverse-probability weighting.
  • An alternative Pediatric Heart Network Z-score system was also used and gave a consistent pattern.

The proximal aorta shows a multisegment architecture in which organization across patients is stronger than within-patient coordination of change.

  • Network findings complement, but do not replace, serial measurement of individual aortic levels.
  • The prognostic value of the network findings requires independent validation.

What This Means

This study re-analyzed data from a large clinical trial of children and adolescents with Marfan syndrome, a genetic condition that can weaken the aorta, the body's main artery. Using heart ultrasound measurements taken over three years in 515 young people, the researcher looked at four parts of the aorta near the heart and how they changed together as the children grew. All parts of the aorta got larger in absolute size, as expected with growth. But when size was adjusted for body size (Z-scores), the values went down slightly, meaning the aorta grew a bit less than expected relative to the children's growth. This drop appeared larger in children aged 6-11 years, though that analysis was exploratory. The study also found that differences between patients in how the aortic segments relate to each other were stronger than the coordination of changes within a single patient over time. This research suggests that looking at the aorta as a connected, multi-segment structure may add information, but it does not replace measuring each part of an individual's aorta repeatedly over time. The author notes that whether these network patterns can predict outcomes has not been tested and needs independent validation.

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Citation

Tirado A. (2026). Longitudinal Remodeling and Network Architecture of the Proximal Aorta in Children and Adolescents With Marfan Syndrome.. Echocardiography (Mount Kisco, N.Y.). https://doi.org/10.1111/echo.70662