This is a long article, and it does require registration to read it, but if you or someone you know is affected by a congenital heart defect, this is a very informative read.
. . . Congenital HD is the most common congenital disorder in newborns, affecting almost 1% of infants worldwide. In countries where children have access to cardiac surgery, 90% of those affected now survive to adulthood. But although many of these patients will continue to suffer complications—heart failure and arrhythmias being the most common—most of them stop having annual cardiac checkups when they reach 18. In most countries, this is the time when they would transition to adult care but, for numerous reasons, the majority simply stops attending the clinic; when they do see a doctor, it is likely to be a primary-care practitioner who knows next to nothing about congenital heart disease.
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Dr Barbara Mulder (Academic Medical Center, Amsterdam, the Netherlands), another leading light in the adult congenital HD (ACHD) movement, concurs. “In the early days of surgery, physicians thought patients were cured. It was never expected that adults would have so many residual defects and that they would develop new problems and have long-term complications,” she explains.
Now Gatzoulis, Mulder, and other thought leaders in the field, along with patient advocacy groups [1,2,3] and national organizations, are stressing the importance of treating congenital heart disease as a lifelong illness. Crucial to this aim is the successful transitioning of these children—beginning in adolescence or even earlier—from pediatric care into the hands of specialists in ACHD. Over half (55%) of affected patients will require long-term care under an ACHD specialist, while the remainder should at least be seen by a regular cardiologist. And the latter should still be assessed at least once in a tertiary ACHD center, to reassure the patient and family and to decide on a path of care, say Gatzoulis and Mulder.
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But, as ever, funding and staffing issues are a huge problem. Even in the countries that perform best in the world for taking care of adults with congenital heart disease, there is a big shortage of medical staff specially trained in this field and a struggle to find enough resources to treat these patients properly. The Netherlands currently leads the way in the care of adults with congenital HD, while the UK, Canada, and some other European countries perform reasonably well. The US, by contrast, lags behind for numerous reasons.
Before surgery for congenital heart disease became the norm in industrialized countries, only around 40% of children born with congenital HD survived, and even just 15 to 20 years ago, survival was, depending on complexity of disease, in the range of 70%. Now almost all children survive in developed countries, and this represents a huge future burden of care.
“This is a looming epidemic,” says Dr Peter Varga (University of Chicago, IL), who works in a mid-sized ACHD unit in Chicago. “In the US alone, there are a million adults living with congenital heart disease, and they have now exceeded the number of children with congenital HD. But existing resources come nowhere near to catering for the vast majority of these adult patients, which means that 90% are either receiving no care or receiving care in a very haphazard way in clinics or primary-care offices that are woefully unfamiliar with these very complex issues.”
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Also key to the disparities between these two North American countries is the nature of funding for healthcare in each place, says Webb. “In the US, business considerations drive everything, and ACHD is near the bottom of the business ladder for cardiology. Things are more organized in Canada. Cardiologists there are more likely to encourage their patients to be followed at adult clinics, whereas American cardiologists will typically be reluctant to allow an ACHD clinic to see their patients—again, business considerations.”
In the US, business considerations drive everything, and ACHD is near the bottom of the business ladder for cardiology.
And Varga explains how the insurance-based healthcare system in the US creates yet another major barrier to the care of adults with congenital HD. “Children here are generally covered up to age 18, either by private or governmental insurance programs, although we do have some pediatric patients who are underinsured or have a hard time availing themselves of care.” But then, at age 18, “All of a sudden they are out the door and they have absolutely nowhere to go: transition time. This has a major impact and is one of the reasons why 90% of the adult congenital HD population falls through the cracks: the fear of not being adequately covered and the reality of not being adequately covered. There are many factors that play a role in this issue, but there is no doubt that insurance is a major one.”
Varga says that patient advocates are working hard to focus attention on this issue, in particular the ACHA. “There is a congressional proposal slowly percolating through the House of Representatives to dedicate resources and attention to adult congenital HD because of the growing impact on the healthcare system in general, but that’s going to take a few years.”
He is cautiously optimistic that healthcare reform may come to the rescue beforehand: “I’m hoping the limited healthcare reform that has been passed in the US is going to have a major impact, because it has a provision about not precluding insurability based on existing disease. And of course all of these people, by definition, fall into this category.
“Ultimately everything boils down to money,” Varga adds. “If Congress dedicated funds to support this effort, I think it would have teeth, it could make an impact. The population of a million adults in the US is growing, because survival is almost approaching 100% into adulthood, and most of these folks are gainfully employed and have a reasonable quality of life, by their own assessment. So outwardly, they are no different than you and me, even with complex heart disease.”
Much, much more at the link.