Basic physical activity in kids with Marfan syndrome slows Aortic Root Dilation

A new study by Elif Seda Selamet Tierney and team found that a basic physical activity intervention in pediatric patients with Marfan syndrome reduced the aortic root (AoR) dilation rate. The findings of this study were published in the Journal of the American Heart Association.

In individuals with Marfan syndrome, stiffer aortas are related with a quicker rate of aortic root dilatation and a higher risk of aortic dissection. Researchers previously demonstrated that modest aerobic exercise lowers aortic stiffness and the rate of AoR dilation in a Marfan animal model. They studied if these findings might be applied to children patients with Marfan syndrome in this study.

24 Marfan syndrome patients aged 8 to 19 years were participated in a 6-month physical activity program, excluding those with ventricular dysfunction or a history of aortic surgery. Patients were told to take 10,000 steps each day, which were measured by an activity tracker. We assessed AoR dimension, endothelial function, arterial stiffness, inflammatory biomarkers, physical activity indices, and coping ratings at baseline and 6 months.

The key findings of this study were:

1.The controls were 15 age matched Marfan syndrome individuals.

2.Twenty-four Marfan syndrome patients were included.

3.The majority of these individuals were sedentary and had abnormal vascular health, according to the baseline examination.

4.The intervention was completed by 22 patients, who walked an average of 7709±2177 steps each day.

5.Patients used their Garmin trackers on 92.8% (IQR, 84%-97%) of intervention days.

6.The rate of change in AoR Z score in the intervention group was substantially lower than in the controls.

This therapeutic intervention in juvenile MFS patients indicated that a basic physical activity intervention of 10,000 steps per day for 6 months was viable in this group and has the ability to reduce the AoR dilation rate. Because the emphasis has been on activity limits rather than exercise promotion for individuals with MFS, our findings may assist alter the paradigm in this patient population. These early findings call for more clinical research to thoroughly assess the influence of exercise, perhaps multiple forms of exercise, on AoR dilation in this patient group, which would most likely be best accomplished through a multicenter randomized exercise intervention.

Reference:

Selamet Tierney, E. S., Chung, S., Stauffer, K. J., Brabender, J., Collins, R. T., II, Folk, R., Li, W., Murthy, A. K., Murphy, D. J., & Esfandiarei, M. (2022). Can 10 000 Healthy Steps a Day Slow Aortic Root Dilation in Pediatric Patients With Marfan Syndrome? In Journal of the American Heart Association (Vol. 11, Issue 23). Ovid Technologies (Wolters Kluwer Health). https://doi.org/10.1161/jaha.122.027598