Abstract

Introduction: Traditionally, patients with heritable thoracic aortic disease (HTAD) have been restricted from competitive sports due to concerns over acute aortic dissection. Patients with HTAD also exhibit lower quality of life (QOL) and physical fitness than general populations. Murine data suggests exercise improves aortic health and does not contribute to aortic enlargement. Hypothesis: Exercise and competitive sports participation will be associated with improved QOL in HTAD patients. Aims: Investigate the effect of lifetime exercise ‘dose’ and competitive sports participation on QOL in patients aged 15-35 with syndromic HTAD [Marfan syndrome (MS), Loeys-Dietz syndrome (LDS), vascular Ehlers-Danlos syndrome (vEDS)] and non-syndromic HTAD. Methods: This cross-sectional study employed a questionnaire to assess lifetime exercise exposure and utilized the PedsQL QOL Inventory. We developed an Exercise Score (ES) to quantify lifetime exercise exposure based on questionnaire results (3 points for competitive sports season, 1 point for recreational sport season. Questionnaires were completed via telephone interview with complimentary medical chart review. Results: Of the forty patients enrolled, the mean age was 26 years. The diagnosis was MS in 82.5%. Despite 87.5% of patients being restricted by their cardiologist, 65% reported competitive sports participation and 92.5% reported some lifetime recreational exercise. The median ES for our population was 19. Participants with an ES greater than the median had a significantly better total QOL score compared to participants with an ES below the median (78 vs 65, p=0.03). There was no difference in aortic size or need for surgical intervention between those above and below the median ES. This also held true for those who did and did not participate in competitive sports. There was a strong positive correlation between the amount of current exercise (days-per-week) and total QOL score (corr=0.55, p<0.001). Conclusions: Increased lifetime exercise exposure and current physical activity levels were associated with improved QOL in young adults with HTAD. There did not appear to be a detrimental effect of exercise on aortic size or need for surgical intervention.

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