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(l-r) Pediatric cardiologist Dr. Thomas Chin and pediatric cardiothoracic surgeon Dr. Jeff Myers consult on a heart surgery patient.

In the dawn of the 21st century, the number of adults with congenital heart disease surpassed the number of children with the disease. That is a direct result of advanced surgical techniques, devices and medication.

Nearly 40,000 babies are born with a heart defect each year, as reported by the American Heart Association (AHA). Twenty years ago, some of the same heart defects that are now easily treated with surgery and medication would have been a death sentence or greatly reduced a child's lifespan. Conditions such as atrial or ventricular septal defects (holes in the heart) that were once treated through a surgical procedure are typically being handled in the cath lab by cardiologists. Cardiothoracic surgeons are now more often than not dealing with the more complex malformations. This overall paradigm shift is encouraging the leaders at Le Bonheur Children's Medical Center along with the cardiac staff to create a more efficient system for keeping up with these children as they transition into adulthood.

"Where we tend to lose kids is in the teen years," said Dr. Jeff Myers, chief of pediatric cardiothoracic surgery at Le Bonheur. "They don't want to come to appointments and then when they get out of the house, they don't come at all.

"You may also have a kid (with congenital heart disease) who's been perfectly fine for 14 years and the thought is he's fine and doesn't need to go to the doctor," Myers continued. "It's hard to take a healthy kid to the doctor; no one sees the reason behind it. It's because the disease is so insidious. You have to monitor how it deteriorates."

Myers and his surgical team handle complex heart malformations, including the most lethal defect, hypoplastic left heart syndrome (HLH). According to AHA, hypoplastic left heart syndrome is an underdevelopment on the left side of the heart, including the aorta, aortic valve, left ventricle and mitral valve. This defect is fatal in the first days or months of life if left untreated. Although HLH is not correctable, babies can be treated through a series of operations in several stages. Over the past 20 years, operations have greatly increased the survival rates of these children, although long-term outcomes are still uncertain. Le Bonheur sees about seven to 10 cases of HLH annually. In 2006, Le Bonheur's pediatric cardiac team saw 386 total inpatients and performed 251 surgical procedures, 132 of which were open-heart.

The causes of heart defects are still unknown. Many children who require surgical repair of heart defects undergo surgery before age 1, according to the March of Dimes. Until recently, it often was necessary to make temporary repairs and postpone corrective surgery until later in childhood. It benefits patients to have early corrective surgery — it can prevent the development of additional complications and allow a child to live a more normal life, sooner. Operations help tremendously but they do have their limitations, which means the heart will deteriorate over time. Part of the reason is the materials used, such as animal or human tissue valves that eventually give out. Another reason is simply the natural evolution of cardiac function; for instance, as a valve leaks over time, the heart stretches and expands, effectively weakening the muscle. While some of these children make it into adulthood with congenital heart disease, they eventually come to heart failure, which is why it is of paramount importance to keep up with patients throughout their lives before the damage intensifies.

In its partnership with the Methodist Healthcare System, Le Bonheur has the ability to ensure patients have the opportunity to stay in the system throughout their lives. Not only can they stay in the system, but they can be tracked by physicians who are adequately trained to deal with congenital heart disease.

"What we consider our mission is that continuum of care, to keep those kids in the system so they can continue to be monitored and followed up so that if they do need surgical care, it's done appropriately," explained Myers. "Failure to do that results in a much larger operation, whether it's transplantation or a bigger operation to repair the damage that's been done."

Currently there are only a handful of adult congenital cardiac programs in the country, mainly due to the fact that children who were operated on in the 1970s and '80s are becoming adults. It has become quite evident that, even in children who are completely repaired as infants, congenital heart disease requires a lifetime of care. The March of Dimes reports that about 1,000,000 people with heart defects are alive today. While they are living longer, children and adults with certain heart defects, even after surgical repair, remain at increased risk for ancillary health problems such as infections involving the heart and valves as well as pulmonary and gastrointestinal problems. Many patients will have to remain on heart medications for life while others will survive on mechanical devices for months or years as they await heart transplantation.

In the coming years, Le Bonheur will work to recruit not only pediatric cardiologists, but cardiologists who will specialize in adults with congenital heart disease. Since children are in the Le Bonheur system until age 18, the advantages of transitioning them into the Methodist system include consistent monitoring, established medical records and physicians who will have a full history of these heart patients.

"Adult cardiologists primarily deal with a set group of diseases and often don't have a real comfort level dealing with adults with congenital heart disease," Myers added. "What we're allowed to do as a healthcare system is integrate that care. Kids can be seen by both adult and pediatric cardiologists and adult and pediatric cardiac surgeons — all available to them within the system."

This union will make it possible in the future to avoid instances of Le Bonheur surgeons having to perform heart surgeries on adults. While it is acceptable to do so, there is an issue with comfort level.

"The non-cardiac issues between adults and children are very different," explained Myers. "The reality is, out staff is very highly trained to take care of kids and problems in children. You want to be able to maximize that expertise and make sure those people are taking care of children."

Myers said fostering a transition program gives the two institutions the opportunity to create an efficient system that ensures these patients don't fall out of it. So if a patient doesn't show up after his 19th birthday, the cardiac staff will know about it.

"It will be a big advantage for us," added Myers. "and it is going to be a big advantage for taking care of these kids in the Mid-South area."

Building a Comprehensive Cardiac Team

Every morning at 7 a.m., cardiologists, cardiothoracic surgeons and intensivists at Le Bonheur meet to discuss each patient who is having heart surgery. Surgeons rely on the cardiologists for detailed, accurate imaging while cardiologists rely on surgeons to make the necessary repairs for a child to have improved heart function. Working together, this cardiac team can reach a consensus for the best solution for each child. This unification was conceived due to the rapid progression of technology and medication along with the equally rapid growth or patient acuity. On top of that, the regional demand for care has increased.

Dr. Thomas Chin, director of pediatric cardiology at Le Bonheur, has been further expanding the cardiology program in the past year across the Tri-State area. He's been working to develop more expertise within each area of cardiology, from interventional catheterization to noninvasive echocardiogram evaluation. There are now an east Memphis location and six satellite offices across Tennessee, Mississippi and Arkansas. The cardiac program is also benefiting from telemedicine hookups to assist rural communities.

"We've been focused on building the inside hospital expertise and specialties but now we're getting to the point where a lot of demand (is coming) from the outside areas to service their needs, so that's where we're headed," said Chin, adding that there has also been a bigger demand from the Hispanic population.

"We have moved from dealing with general cardiac problems to dealing with more and more complex patients," Chin explained. "It's not just volume, but the complexity of it. That's when you start needing a separate cardiac ICU, much more than just a physical space where we can house the cardiac patients, but developing the expertise to deal with patients."

In September, Le Bonheur will officially open a cardiovascular intensive care unit (CVICU), distinctive from the general ICU. A CVICU is part of Le Bonheur's bigger plan to integrate all cardiac services. The new, six-bed unit is being renovated from the existing ICU. When the new hospital is completed around 2012, the CVICU will be in an independent space housing 10 beds.

This concept has evolved along with the general notion that ICU cardiac patients require specific, focused care above and beyond general critical care needs. A CVICU offers cardiac patients the benefit of specialized expertise along with a comprehensive team of physicians who can handle a patient from imaging to diagnosis to surgery.

"Taking care of (cardiac) patients in one place where you have carefully defined nurses and physicians who see a higher number of patients provides a more specialized care model for these patients," Myers explained. "Both in adult and congenital cardiac surgery, there is pretty good evidence that the outcomes are better when the patients are cavorted like this."

Another aspect of the cardiac integration is the surgery tag team or hybrid OR, where surgeons and cardiologists work in tandem on patients; for instance, cardiologists would close a hole in the heart with a catheter before the surgeon begins reconstruction. This has the ability to minimize the negative parts of an invasive procedure.

"Part of the operation is done by cardiologists and part of it is done by cardiothoracic surgeons," explains Myers. "Patients spend less time on a heart/lung machine because you don't have to stop the heart to do the operation that's inside the heart. Surgeons can just do their part on the outside and cardiologists can do their part on the inside. We've done that several times now and it's been very successful."


February 2007

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