When Did Noah Build the Ark?

In the adult congenital heart disease (ACHD) community, two important studies were recently published that highlight an emerging and growing niche—that of advanced heart failure in adults with CHD:

• “Improved Outcomes of Heart Transplantation in Adults with Congenital Heart Disease Receiving Regionalized Care,” by researchers at the University of Washington.
• “Center Volume and Post-Transplant Survival Among Adults with Congenital Heart Disease,” by researchers, including myself, at Vanderbilt University.

As you well know, due to the innovative treatment options for those born with congenital heart disease (CHD), there are now more adults with CHD than children. As this population ages, they develop heart failure, which has become the leading cause of death for adults with CHD. While we still have limited data on best practices and options for these patients, now is an exciting time for us as a community to improve this.

My excitement may be biased (keep reading anyway)—I’m a transplant cardiologist at Vanderbilt who focuses on adults with CHD who develop heart failure and need advanced therapies such as mechanical pumps (ventricular assist devices, or VADs) or heart transplantation.

The key to capitalizing on this opportunity lies in this blog title, “When Did Noah Build the Ark?”… BEFORE the flood. Our ACHD patients need us to be prepared to care for them as they develop heart failure and require advanced therapies. And we must do so in a way that gives our patients the best possible outcomes and prepares them for the possibility of needing a transplant.

The study from the University of Washington by Dr. Vidang P. Nguyen and colleagues, published in the Journal of the American College of Cardiology, was an evaluation of nearly 2,000 ACHD patients that were listed for heart transplants between January 2000 and June 2018. Their findings, while not surprising, are essential as a basis of reference. Let’s focus on two key aspects:

1. ACHD patients who are waiting for transplant have a lower risk of death if they are being followed at Adult Congenital Heart Association (ACHA) accredited centers.

For starters, it’s important to remind patients and providers around the world that each ACHD heart and story is unique in its development, surgical repair, and treatment. Because of this, it is essential that ACHD patients are cared for by doctors and nurses who specialize in caring for this population. A quick analogy—as an NYC guy who has embraced all things Nashville, I think I can confidently say that just because you make the world’s best bagels, doesn’t mean you can compete in the world of hot chicken.

Beyond ACHD experts, what specifically do these centers offer? “[They]... are required to have a multidisciplinary team with multiple board-certified ACHD cardiologists and must include dedicated ACHD heart failure specialists (like me), congenital heart surgeons, and comprehensive diagnostic and interventional services by specialists in CHD.”

It truly takes a multidisciplinary collaborative team to be successful. To see who’s involved, I’d point you to a graphic demonstrating the necessary teams required that we included in another recent publication, “Advanced Heart Failure in Adults with Congenital Heart Disease.”

OK, so let’s agree that the more specialized and collaborative care one has prior to transplant, the better they do. But what about the actual transplant surgery and post-operative management?

This brings us to the second important point:

2. Post-transplant survival was improved at the highest volume centers. In our study published in the Journal of Heart and Lung Transplantation, we proved that those centers that complete 14 or more transplants (ACHD and non-ACHD) had significantly better outcomes than those who did less. Then, the evaluation by Nguyen showed that the transplant centers that do the most ACHD transplants have the best outcomes. Again, we should not find this surprising. Practice makes perfect.

It is our responsibility as providers to make sure that our patients are treated at the centers most capable of providing successful outcomes. But what burden lies with patients? Truth be told, for patients and their families, the idea of transplant is scary. This is completely understandable, but not talking about it doesn’t make heart failure improve.

Remember the Ark—being prepared is key. As patients and families go through this process, I encourage you to ask questions, use your resources to be informed, and importantly, become the teacher as well. It’s true that ACHD patients have complicated hearts and histories, but they also have fascinating stories of overcoming adversity, taking risks in the absence of data, and surviving.

We want and need to hear about your history, and we are excited to work to become part of your future as well.