Aortic Z-scores fall over 36 months in pediatric Marfan syndrome despite diameter increaseNew analysis reveals how aortic growth patterns affect Marfan syndrome

Echocardiography (Mount Kisco, N.Y.) Published November 4, 2026 Study authors: Tirado Alberto Guevara PubMed ↗ DOI ↗ Editorial oversight: Dr. Sofia Müller, MD · Lifespan & Whole-Person Care

AI-generated summary of the cited source, checked by automated accuracy review. How we work

Key Takeaway

Consider that aortic Z-scores may decrease over time in children with Marfan syndrome despite rising diameters.

This secondary analysis of a randomized trial examined longitudinal remodeling and network architecture of the proximal aorta in 515 children and adolescents with Marfan syndrome over 36 months. Of these, 445 participants had five acceptable visits. The study monitored proximal aortic dimensions and derived network measures; no comparator was reported.

Absolute diameters of aortic segments increased from baseline to 36 months. In contrast, Z-scores of aortic segments decreased over the same period, with effect sizes ranging from -0.688 to -0.454 (p < 0.001). Interindividual network strength was stronger than intraindividual network strength (1.783 vs 1.020; difference 0.763; 95% CI 0.526-1.101; p < 0.001).

Safety and tolerability data were not reported. The analysis was descriptive, and the prognostic value of the network findings requires independent validation. No funding or conflict of interest information was provided.

The findings suggest that network-based measures may complement serial measurement of individual aortic levels, but their clinical utility remains uncertain. Clinicians should continue to rely on established monitoring strategies until further validation is available.

How this fits prior evidence

Prior coverage highlighted that multi-factor management models for thoracic aortic disease improve precision beyond diameter-only assessment. This analysis extends that concept by describing network architecture of the proximal aorta in pediatric Marfan syndrome, showing that interindividual network strength exceeded intraindividual strength. The observed decrease in Z-scores alongside increasing absolute diameters underscores the limitation of diameter-only monitoring and supports consideration of additional factors, though the prognostic value of these network measures requires independent validation.

Living with Marfan syndrome means constant monitoring of the aorta, the main artery leaving the heart. For children and teens, doctors usually track the size of this vessel to manage risks. A new analysis of a trial involving 515 young patients looked deeper into how these measurements change over time.

The study found that while the actual size of the aorta increased over 36 months, the way different segments of the artery connect and relate to one another stayed more consistent. These network patterns were stronger than the changes seen within a single person's measurements over time. This suggests that looking at the aorta as a connected network might provide a clearer picture of how the artery is changing.

While these findings are promising, the researchers noted that we still need more independent testing to know exactly how well these network patterns can predict future health issues. For now, these findings offer a new way to complement the standard measurements doctors already use to monitor patients.

What this means for you:

Mapping the aorta as a connected network may provide more consistent data than tracking single measurements alone.

Common questions

What did the study find about the size of the aorta?

The study looked at 445 participants who had five acceptable visits. It found that the absolute diameters of the aortic segments increased from the start of the study to the 36-month mark.

How does the network approach differ from standard measurements?

Standard measurements look at individual segments. This study found that the interindividual network strength was stronger than the intraindividual network. This means the connections between different people's measurements were more consistent than the changes seen in one person's measurements over time.

Is this a proven way to predict health risks?

The study is still in the early stages of evaluation. The researchers noted that the prognostic value of these network findings still requires independent validation before they can be used to predict specific outcomes.

Study Details

Study typeRct

Sample sizen = 515

EvidenceLevel 2

Follow-up216.0 mo

PublishedNov 2026

PMID42977707

View Original Abstract ↓

BACKGROUND: Aortopathy is the main cardiovascular manifestation of Marfan syndrome, but the joint evolution of the different segments of the proximal aorta during pediatric growth remains insufficiently characterized. OBJECTIVE: To evaluate longitudinal remodeling and the interindividual and intraindividual architecture of the proximal aorta in children and adolescents with Marfan syndrome. METHODS: This was a secondary analysis of data from the Pediatric Heart Network Marfan trial. Participants aged <18 years at randomization with acceptable echocardiograms obtained at baseline and at 6, 12, 24, and 36 months were included. The aortic annulus, sinuses of Valsalva, sinotubular junction, and ascending aorta were analyzed using absolute diameters and Z-scores. Partial correlation networks were estimated, separating interindividual and intraindividual components. Uncertainty was assessed using participant-level bootstrap resampling. RESULTS: A total of 515 participants were included; 445 (86.4%) had five acceptable visits. From baseline to 36 months, all absolute diameters increased, whereas Z-scores decreased (-0.688 to -0.454; all p < 0.001). Exploratory age-stratified analyses showed larger decreases among participants aged 6-11 years. The interindividual network was stronger than the intraindividual network (global strength 1.783 vs 1.020; difference 0.763; 95% CI 0.526-1.101; p < 0.001). Sensitivity analyses using inverse-probability weighting and an alternative Pediatric Heart Network Z-score system showed the same overall pattern of declining Z-scores. CONCLUSIONS: 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, and their prognostic value requires independent validation.

Aortic Z-scores fall over 36 months in pediatric Marfan syndrome despite diameter increaseNew analysis reveals how aortic growth patterns affect Marfan syndrome

How this fits prior evidence

Common questions

Study Details

Evaluating Text Message Video Interventions for Improving Infant Sleep Safety Practices

Text message videos for safe sleep show mixed results

Clinical research that matters. Delivered to your inbox.

Aortic Z-scores fall over 36 months in pediatric Marfan syndrome despite diameter increaseNew analysis reveals how aortic growth patterns affect Marfan syndrome

How this fits prior evidence

Common questions

More on Marfan syndrome

Study Details

Evaluating Text Message Video Interventions for Improving Infant Sleep Safety Practices

Text message videos for safe sleep show mixed results

Clinical research that matters. Delivered to your inbox.

Related in Pediatrics

From Other Specialties