Детская больница в Лос-Анджелесе внедрила стратегию «катетеризация перед операцией» для спасения новорожденных с тяжелой формой порока сердца

Newswise — A Children’s Hospital Los Angeles team has developed a new “catheter first” approach for managing newborns with the most severe heterotaxy-associated congenital heart disease. The minimally invasive strategy grew out of the team’s 217-patient study—the largest published analysis of its kind—which found that fewer than half of the highest-risk infants with heterotaxy survived their first heart surgery. John David Cleveland, MD, Associate Chief of Cardiothoracic Surgery in the Heart Institute at CHLA—home to one of the highest-volume heterotaxy programs in the world—explains what clinicians learned and how those insights changed their practice. What did your study reveal about the highest-risk newborns with heterotaxy syndrome? We found that the standard pathway was too often not working for babies with the most severe disease. These are newborns with single-ventricle heterotaxy, obstructed total anomalous pulmonary venous return (TAPVR), and pulmonary arteries that are not connected to the heart. The standard paradigm is that once they’re born, you rush them to the operating room to repair the lesion. But we found that fewer than 50% survive that operation, and even fewer continue down the single-ventricle pathway in a good fashion. This is a group that needs a new approach. How did you change the way you treat neonates with single-ventricle heterotaxy and TAPVR? We worked with our interventional cardiology colleagues to develop a different upfront strategy. Instead of rushing them straight to surgery, we now use catheter-based palliation first to stabilize them. In the cath lab, we place stents in the vessels involved in blood flow to and from the lungs. The goal is to buy these babies time—time to grow, get stronger, and become better candidates for that first operation. What have you seen so far with this catheter-first approach? We’ve used this approach in four patients so far, and all four have survived. That’s a very small number, but in a group where survival has historically been extremely poor, it’s encouraging. In one case, we used staged catheter-based palliation to stabilize a newborn who had an extremely high risk of mortality. By performing two cath procedures in the first month of life—and a third at 2 ½ months—we were able to delay that first surgery until 3 ½ months of age. That child is now 7 months old, at home, and doing well. And because the first operation was delayed, we hope to delay the Glenn procedure until around 9 to 12 months of age. What other lessons emerged from your study? One key finding was that biventricular patients do significantly better than single-ventricle patients. So when there’s a door open for biventricular repair, we make a real effort to walk through it. That’s been another key change in our management paradigm. On the transplant side, we found that once these children start needing procedures outside the standard pathway—especially atrioventricular (AV) valve surgery or Fontan revision—the odds of meaningful improvement go way down. It’s often better to refer for transplant early rather than continue high-risk interventions. Why should babies with severe heterotaxy-associated heart disease be referred to a high-volume center? Heterotaxy is incredibly variable, and care is highly individualized. If you’re seeing one or two of these patients a year, that’s very different from a center like CHLA, where we see more than one a month. This is a complex condition our entire team knows how to manage. That care starts before birth. Our Fetal Cardiology team, interventional cardiologists, surgeons, and other specialists are all involved early, so we can build the right plan from the beginning. That kind of experience gives these very high-risk children the best chance to not only survive, but to thrive.