Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect in which the left side of the heart is severely underdeveloped. The care for patients with HLHS is complicated and requires multiple surgeries in infancy and sometimes into adulthood. Studies have consistently confirmed that when an institution performs high volumes of a complex procedure, their outcomes are significantly improved compared to low-volume institutions.
The information provided below is designed to help you and your family choose the right hospital for the type of HLHS care you need. Although choosing a hospital or provider may be impacted by insurance or finances, there are some important points to keep in mind.
The biggest distinction among pediatric cardiac surgery centers is the sheer number of patients they treat every year. The reporting data typically combines all cardiovascular surgeries performed on patients under age 18. However, only a minority of those cases are the complex surgeries that patients with HLHS need, such as the Norwood and Glenn. Because of the importance of having a surgeon and team who are highly experienced at HLHS surgeries, it’s crucial to find a center that routinely performs a high number of those procedures.
Decades of research has shown that having a few surgical centers that each specialize in different procedures leads to significantly better outcomes. This is because it allows surgeons and care teams to focus on the needs of a specific patient population instead of performing a broad range of procedures.
For example, in 1993, Sweden centralized its process for pediatric cardiac surgery by only allowing the top two hospitals with the lowest surgical mortality rates to operate. Within two years, their national average of surgical mortality dropped from 9.5% to 1.9%. This significant improvement also happened over a period of time when the centers were performing more complex surgeries than before.
Similarly in the United States, a review of over 32,000 patients across 48 centers found that low-volume centers had an average mortality rate of 14.8% for complex cases, compared to a significantly lower rate of 8.4% at the high-volume centers. Additionally, Norwood mortality rates at low-volume centers were 36.5%, compared to 16.9% at high-volume centers. All centers performed the same for low-difficulty operations. In other words, high-volume programs have lower mortality rates than low-volume ones when performing complex surgeries.
In order for patients and families to make informed decisions about where to get care, they need to have access to honest, reliable information. Hospitals must be transparent about their outcomes not only to reassure patients that they are safe, but also so hospitals themselves can focus on improvement.
The National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) has been working with many centers to successfully implement quality improvement and transparency practices. However, they note that there are still many barriers, “including health care organization’s concerns about potential negative effects of disclosure on reputation and finances, and lack of reliable definitions, data, and reporting standards for fair comparison of centers.”
“Transparency – sharing data or information about outcomes, processes, protocols, and practices – may be the most powerful driver of health care improvement.” ~NPCQIC, 2015
The Pediatric Congenital Heart Association (PCHA), the largest congenital heart advocacy organization in the United States, published their Key Tenets of Transparency in 2015. Suggestions for transparency include publicly reporting outcomes data, having a formal review process that regularly assesses performance, and providing families with a list of questions to facilitate transparent discussions.
Of note, the PCHA recently released a statement noting their concern with how US News & World Report rankings are calculated and presented.
The rankings appear to inflate centers that perform fewer complex procedures, ranking them higher than centers that frequently take on the riskier patients… At face value, these rankings do not allow families to consider such relevance to their child, particularly failing those families of children with complex congenital heart defects.
The concerns of the PCHA further highlight how vital it is for hospitals to provide the transparent data that families and patients need to make an informed decision.
As children with HLHS age, their medical and surgical needs change. Older patients may need valve replacement, re-replacement, or heart transplant. Just like in the early stage surgeries, patients should look for hospitals that perform more of these procedures, and providers who specialize in managing HLHS in older patients.
The concept of “Centers of Excellence” has been developing in the health care field. It’s the idea that specialized programs can focus their efforts to provide exceptional care above and beyond general all-around centers. This kind of approach in HLHS care means that certain hospitals would become experts and excel at one aspect of HLHS care (ex., Norwoods and Glenns) while other centers excel in other areas (ex., transplant). Some hospitals have recently begun partnering with others nearby to reduce competition and develop local Centers of Excellence.
Regardless of what stage of HLHS care you or your child need, the most important thing to remember is this: High Volume = High Quality. You can find a list of questions the HLHS Program suggests asking at various stages of treatment on our website. Your provider should be willing to take the time to have an open, honest discussion with you – and you should advocate to get the information you need to feel comfortable and make an informed decision about HLHS care.
The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of finding solutions for individuals affected by congenital heart defects including HLHS. Our specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies. Email us atHLHS@mayo.edu to learn more.