Commentary: Can we ever “cure” aortic valve disease in children?

The group from Melbourne presents a retrospective study spanning 36 years on primary aortic valve (AoV) repair performed in children (23% neonates, 29% infants). The primary goal was to compare results in the cohort that resulted in a bicuspid valve configuration with those that resulted in a tricuspid configuration. They conclude that repair strategies resulting in a bicuspid valve yields satisfactory outcomes. The bicuspid group had excellent long-term survival, and a freedom from AoV reoperation at 15 years of 32%. Changing valve morphology into tricuspid anatomy did not offer any advantages, and was maybe disadvantageous. They therefore conclude that if a bicuspid repair is feasible, this should be favored.

Although not new, the core message, that a repair leading to a bicuspid valve can function well, is important. I will note that not everyone agrees that a bicuspid repair is favored over a tricuspid repair. With regard to pediatric AoV disease in general, the Melbourne group is well known for their advocacy of primary AoV repair in neonatal critical aortic stenosis (AS) over balloon valvuloplasty. Their other clinical stance is in favor of primary AoV repair under almost any circumstances, reserving a Ross procedure for later, if necessary. Here, I will insert the caveat that none of these management practices, including ours (which differs from theirs), have been subjected to anything better than retrospective mostly single-center studies, with regression analyses and propensity matching improving the clinical significance somewhat. This could also be called “informed speculation.” The best way to manage pediatric AoV disease is so highly dependent on institutional experience, technical capabilities, and anatomic and clinical variations that I suspect this particular debate will go on for a long time. One might argue that critical AS should not be managed as an all-or-nothing approach, and should be discussed in a multidisciplinary fashion. Some patients are probably better off with a primary transcatheter approach (those with larger aortic annuli, less cusp pathology), whereas some do better with a surgical approach (more diseased valves with smaller annuli). However, the downside of such an approach is that surgeons operate on more advanced pathologies in general, and thus will have less opportunities to apply so-called “simple” repair techniques (no patch use, commissurotomy, and shaving of cusps), such as depicted in the beautiful video that accompanies the work from Schultz and colleagues. Our “simple” repair cohort, for example, is one that typically includes other surgical interventions, typically aortic arch repairs, thus making a balloon valvuloplasty not logical. We would also take issue with the strategic goal of delaying the Ross procedure to a second or third procedure, especially if a complex repair is used at the time of the first procedure. I am not sure I see the logic in that, because reoperating on the right side for a conduit change is usually safer than reoperating on the left side, when doing a Ross after 1 or 2 failed valvuloplasties. In final analysis, we prefer to think of the best therapeutic strategy as the one that results in not only fewer reinterventions, but in the best possible milieu for the left ventricle in which to operate.

Especially if there is mixed AS and aortic insufficiency after an AoV repair, and even though a reintervention might be pushed to years away, the left ventricle will suffer.^, Conversely, seeing the many teenagers with normal aortic roots after infant Ross, one can't help but think of the early Ross as being a better operation. In our hands, aside for patients who can be helped with simple AoV repairs, a well-executed Ross procedure, especially in young patients, remains the closest we can offer to a “cure” for the left side.