Echocardiography. 2026 Nov;43(11):e70662. doi: 10.1111/echo.70662.
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.
PMID:42977707 | DOI:10.1111/echo.70662
