Key result
Pediatric Marfan syndrome patients show preserved cardiac reserve and vascular adaptability during exercise despite baseline abnormalities.
Why the study?
Pediatric Marfan Syndrome is associated with vascular and ventricular changes, and resting measurements may not fully capture cardiovascular functional reserve detectable by exercise stress echocardiography.
Does exercise stress echocardiography reveal impaired cardiovascular functional reserve in pediatric patients with Marfan Syndrome compared to age-matched controls?
Does exercise stress echocardiography reveal impaired cardiovascular functional reserve in pediatric patients with Marfan Syndrome compared to age-matched controls?
Pediatric Marfan Syndrome patients exhibit preserved cardiovascular reserve during exercise despite baseline vascular and ventricular abnormalities, supporting their safe participation in endurance activities.
Reassures clinicians on exercise tolerance in pediatric Marfan syndrome; leaves open sports guidance pending larger prospective trials.
Abstract Background Pediatric Marfan Syndrome (MFS) is associated with changes in aortic stiffness, endothelial function, and ventricular performance. Resting measurements may not fully capture cardiovascular functional reserve. Exercise Stress Echocardiography (ESE) can reveal subclinical impairments not evident at rest. Methods This study included 31 pediatric MFS patients and 31 age-matched controls. Resting and exercise echocardiography were performed to assess systolic and diastolic function, aortic dimensions, and vascular parameters. Results MFS patients had larger baseline height and BSA values compared to controls. Significant baseline differences in aortic dimensions, diastolic function, endothelial function, and vascular stiffness were observed in MFS patients. Despite these differences, MFS patients demonstrated preserved systolic and diastolic reserve during exercise. Vascular responses to exercise were comparable to controls, reflecting increased stiffness and altered endothelial function but preserved adaptability. Conclusions Preserved cardiovascular reserve during exercise suggests baseline abnormalities may not result in significant dysfunction, supporting safe participation in endurance activities for pediatric MFS patients.
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Khodabakhshian et al. (2026) studied this question. Pediatric Marfan Syndrome patients showed preserved systolic and diastolic reserve and vascular adaptability during exercise despite baseline aortic and vascular abnormalities.
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