By Michael H. Crawford, MD, Editor

SYNOPSIS: If they follow recommendations for restriction from competitive sports, more than one-third of children and young adults with isolated bicuspid aortic valves could be disqualified mainly for mild aortic dilatation.

SOURCE: Baleilevuka-Hart M, Teng BJ, Carson KA, et al. Sports participation and exercise restriction in children with isolated bicuspid aortic valve. Am J Cardiol 2020;125:1673-1677.

In 2015, the American Heart Association/American College of Cardiology (AHA/ACC) updated their recommendations for participation in competitive sports for school age children and young adults. However, there is a paucity of data on the effect of these recommendations on patients with isolated bicuspid aortic valve (BAV) disease. Baleilevuka-Hart et al performed a retrospective, longitudinal analysis of the Johns Hopkins Pediatric Cardiology Database of patients age 5 to 22 years with isolated BAV from 2000 to 2013. They excluded patients with symptomatic cardiovascular disease, other congenital anomalies, prior aortic valve intervention, Kawasaki disease, and pregnancy.

Of the 345 patients identified, 17 had incomplete echocardiographic data and 202 already were restricted from sports because of aortic dilatation. This left 123 patients with no restrictions regarding athletic participation according to the recommendations from AHA/ACC. Investigators used a binary approach (restricted from competitive sports or not) based on the following criteria for restriction: peak aortic gradient > 40 mmHg, aortic regurgitation > moderate, evidence of aortic root dilatation, ascending aortic dilatation, enlarged left ventricle (LV), or reduced LV systolic function per the guidelines. Aortic dilatation was described by diameter measurements corrected for body surface area (Z score). Survival was truncated at six years of follow-up; median follow-up was 4.4 years.

Over the course of follow-up, 36% of patients met criteria for sports restriction, and about one-third met more than one criterion. However, no adverse events or deaths were recorded. The most common cause for restriction was aortic dilatation, which was found in 34% of patients, and it generally was mild (Z score, 3-3.9). Significant aortic stenosis developed in 2%, and > moderate aortic regurgitation developed in one patient. The authors concluded that if the new recommendations were followed, more than one-third of young patients with isolated BAV would be restricted from competitive sports mainly for mild aortic dilatation.

COMMENTARY

On one hand, this may seem like a no brainer: If a child or young adult with isolated BAV meets any sports restriction criteria, they should be disqualified to prevent a serious cardiovascular event. However, as this analysis of a pediatric to young adult database shows, such a policy would restrict more than one-third of these young people from participating in competitive sports, mainly for mild dilatation of the proximal aorta. Is this reasonable?

First of all, this would involve a lot of youngsters, since BAV is one of the most common congenital heart defects. Also, other studies have shown mixed results on the effect of athletic activity on aortic size over time. Any increase was mild and gradual. In addition, large follow-up studies have shown tiny rates (or zero) aortic dissections in young people with BAV. In the Baleilevuka-Hart et al study, 7% of patients who met disqualification criteria failed to meet them on the next echocardiogram. This could be because of measurement error or the vagaries of correcting measurements for body size and growth. Finally, disqualification for sports participation may psychologically harm children and young adults, which could affect them the rest of their lives.

This analysis contains weaknesses, including the fact it was a retrospective chart review study. The authors could not assess the effect of sports participation because it was not well documented. However, their data raises the potential negative effect of strict adherence to the AHA/ACC recommendations. Considering the paucity of data to support disease progression and cardiac events caused by sports participation, the authors recommended a thoughtful approach to applying the guidelines.