Wednesday, 31 August 2011

Comment: Children's heart surgery under threat

Dr Duncan Macrae, director of children's services, Royal Brompton & Harefield NHS Foundation Trust and president of the Paediatric Cardiac Intensive Care Society.
Dr Duncan Macrae, director of children's services, Royal Brompton & Harefield NHS Foundation Trust and president of the Paediatric Cardiac Intensive Care Society.

Friday, 26 August 2011 12:20 PM

One in every 133 children is affected by congenital heart disease. Royal Brompton's children's heart surgery unit is threatened with closure under national plans for this specialist service, unless an upcoming judicial review can save it.
By Dr Duncan Macrae
"For all who attend or visit the children's wards it is clear that it is a place of excellence…you feel this as soon as you walk in the door and it is an example of what all hospitals should be like…Children deserve the best and the children's heart surgery services at Royal Brompton are the best."
A parent's comments conjure up a vivid picture of life on the children's wards at Royal Brompton. Many more have expressed similar feelings for the care they receive here, a commitment reinforced by plans to stop paediatric cardiac surgery at the hospital. It is these views which have propelled me and hundreds of other staff at the hospital to do everything in our power to protect our services for children.
The decision by Royal Brompton & Harefield NHS Foundation Trust to launch legal action against the Joint Committee of Primary Care Trusts (JCPCT) follows proposals to discontinue children's heart surgery at Royal Brompton. These follow a national review on the future of paediatric cardiac surgery in England.
We believe that the recommended options put to public consultation are fundamentally flawed, such that a consultation based on them is unlawful. Four options for the reconfiguration of paediatric cardiac services have been put forward. None include Royal Brompton. This is despite the hospital ranking joint-fourth out of all eleven children's cardiac surgery centres in England. Ours is the largest centre for the life-long care of adults and children born with heart defects, with four cardiac surgeons undertaking over 400 cardiac operations each year – criteria required by the national review to ensure the service is safe and sustainable.
Ruling on the trust's legal action in July, Mr Justice Burnett granted permission for the judicial review on all grounds advanced. This will be the first time one NHS body takes another to judicial review. It is a decision taken with great regret, but one which had to be taken knowing our patients have asked us to do everything in our power to protect the services we provide.
If children's heart surgery stops, this puts all our services for these patients under threat. Our paediatric intensive care unit and anaesthesia will immediately become completely unworkable, in turn threatening the future of our highly specialised children's services. They include services for patients with lung diseases such as cystic fibrosis and Duchenne muscular dystrophy and very severe asthma. These are delivered by clinical teams known throughout the world for their expertise. The impact on patients caused by the break-up of our expert teams and the impact of the proposed changes on the long-term financial health of the Trust would, I firmly believe, be hugely detrimental and have not been adequately considered.
As one patient's relative put it: "Closing down a highly successful unit when the capital is struggling to cope with patients seems unwise, unsafe and unsustainable."
The JCPCT have convened an expert panel to consider the knock-on effects of removing children's heart surgery from Royal Brompton. While we welcome this belated move, it is alarming that their terms of reference are very narrow. I believe this is a huge mistake which ignores the 360 degrees of wrap-around care which we offer heart and lung patients of all ages.
I have seen how the national review and JCPCT recommendations have created uncertainty and anxiety among some patients. We are singularly focused on doing everything to reassure patients and families that we will not sit back and allow the care we offer them to be downgraded or adversely affected in any way. We are continuing to invest in our paediatric facilities. We greatly regret the time and resource that is being spent dealing with the aftermath of the JCPCT's proposals. But the commitment of staff and the strength of feeling among patients and families is unequivocal. We have to protect children's heart surgery and paediatric services at Royal Brompton.
Dr Duncan Macrae, director of children's services, Royal Brompton & Harefield NHS Foundation Trust and president of the Paediatric Cardiac Intensive Care Society.
The opinions in's Comment and Analysis section are those of the author and are no reflection of the views of the website or its owners.

Saturday, 20 August 2011

Congenital Heart Disease

content developed with:
Historically, only a minority of children born with a congenital heart defect survived into adulthood; however, recent advances in surgical treatments and techniques have reversed these statistics. Now, approximately 90 percent of children born with a heart defect live healthy lives through adulthood. This shift in survival rates has produced a relatively new group of patients with a new set of challenges for the medical community—adults with congenital heart disease. Today, adults with congenital heart disease outnumber children with the condition.
Although repairs of a heart defect are corrective, they are not curative. All survivors have an increased risk of developing heart problems as their hearts and bodies age. If you have a complex defect, you have an increased risk of heart failure, stroke, and developing heart rhythm problems and/or heart infections. People with complex defects like valve atresia, tetralogy of Fallot, transposition of the great arteries, or abnormal ventricles need to be evaluated in a specialized center for adult congenital heart disease on a regular basis. Regardless of the complexity of the defect, most people who actively manage their congenital heart disease have few or no ongoing symptoms or limitations on what they can do physically.
Most specialists feel that the single most important thing individuals with congenital heart disease can do is learn about their condition so that they can effectively manage it. No adult should rely on parents, a primary care doctor, or anyone else to manage his or her disease.
This section contains information on:
Staying healthy
How well the heart compensates for structural abnormalities is, in part, determined by overall physical fitness and health. People with congenital heart disease need to fully embrace a lifestyle that promotes fitness and heart health.
Key guidelines include:
  • Get regular aerobic exercise as prescribed by a physician.
  • Don't smoke.
  • Don't use street drugs such as cocaine and methamphetamine.
  • Follow a heart-healthy diet.
  • Practice techniques for reducing and managing stress.
When to see a doctor
People with congenital heart disease need to adopt a team approach to manage their disease effectively. The team usually consists of three to five people in addition to themselves:
  • A primary care physician (a family practitioner, pediatrician, or internist)
  • A cardiologist specializing in congenital heart defects (who may be located in a specialized center)
  • Possibly a local cardiologist, if the congenital cardiologist is far away
  • A parent, spouse, or companion
Additional members of the team rotate in and out over time and may include a cardiac surgeon, general surgeon, gynecologist, obstetrician, or genetic counselor. Open communication among team members is fundamental to successfully managing congenital heart disease.
Many aspects of your lifestyle will need to be discussed with the healthcare team, including potential effects of over-the-counter medications, vitamins, herbal preparations, or prescription medications on the heart. You'll also want to discuss potential interactions between any drugs you might be taking for your heart defect and any of the above.
It is important to communicate any changes in your condition or lifestyle to your physician. Fever or other signs of infection, increased fatigue, and shortness of breath all are reasons to contact a physician. Changes in the circulatory system resulting from corrected heart abnormalities can develop over a long period of time, so the first manifestations of these can be very subtle. When in doubt, get it checked out.
It's important for people with heart defects to get regular checkups. The frequency and components of these checkups will vary according to individual circumstances, depending upon your defect(s), age, and overall health. These checkups may include blood tests, standard electrocardiogram, 24-hour ambulatory electrocardiogram, chest X-ray, echocardiogram, or exercise testing.
Children. Periodic heart checkups with a pediatric cardiologist are necessary to evaluate the progression of the condition or verify the continuing success of the treatment. The frequency of checkups depends upon complexity of the defect and treatment plan. More frequent checkups are necessary just after diagnosis, around surgery, and for children with complex defects.
Adults. The frequency at which one sees a cardiologist with expertise in congenital heart disease should be determined on a case-by-case basis by one's health team. For example, a healthy adult with a simple defect, like a small ventricular septal defect or atrial septal defect, may need to see the specialist only once every five years. A more complex defect may require annual consultations. In either case, it is important for proper management of your condition to find a congenital cardiologist who will be available to consult with you and your local physician as necessary. If you have complex heart disease, you should not wait until you have symptoms; you should see your congenital cardiologist on a regular basis.
Endocarditis is a dangerous infection of the endocardium, the lining of the inside of the heart's four chambers. Certain bacteria and other microorganisms that occasionally circulate in the blood may settle and multiply on the heart valves. Abnormal heart valves, where blood flow is turbulent, are especially vulnerable to this kind of infection. Although effective treatments are available, endocarditis can lead to permanent damage of the heart valves or even heart failure.
Most congenital heart defects are associated with increased susceptibility to endocarditis. Individuals at high risk include those who have had endocarditis before, had previous defects in their heart valves or have mechanical valves (such as for aortic valve stenosis), as well as individuals with complex heart abnormalities like single ventricles, tetralogy of Fallot, or transposition of the great arteries. People who have had surgery to construct systemic pulmonary shunts or conduits also are at high risk.
Endocarditis is diagnosed using a combination of blood culture and special echocardiogram. If a child or an adult with congenital heart disease has a fever that cannot be attributed to a specific infection like strep throat or an ear infection, endocarditis should be considered.
Treatment of endocarditis may require four to six weeks or more of intravenous antibiotics and may include surgery. Penicillin (amoxicillin/ampicillin) and gentamicin are the most commonly used antibiotics. Other antibiotics can be used as well: vancomycin, cephalosporin, clindamycin, or azithromycin. Although powerful antibiotics are available, treatment can be difficult and complications of endocarditis may occur.
In order to minimize the risk of developing endocarditis, some people with congenital heart disease will need to take antibiotics prophylactically, prior to certain dental procedures. In some cases the antibiotics will be administered intravenously and may include follow-up doses after the procedure. In other situations, the antibiotics are taken orally, one hour before the procedure. Detailed information on endocarditis treatment can be found on the American Heart Association website.
It's important to note that most cases of endocarditis are not caused by and do not directly follow a dental procedure. More often, the endocarditis follows a minor gum injury or is caused by poor dental hygiene. Regular and comprehensive dental care is imperative for people with congenital heart disease.
Dental procedures requiring endocarditis prophylaxis include:
  • Routine teeth cleaning at the dentist's office
  • Dental extractions
  • Periodontal procedures including surgery, scaling, and root planing, probing, and recall maintenance
  • Endodontic (root canal) instrumentation or surgery
  • Subgingival placement of antibiotic fibers or strips
  • Initial placement of orthodontic bands but not brackets
  • Intraligamentary local anesthetic injections
  • Prophylactic cleaning of teeth or implants
Individuals can do several things daily to guard against an endocarditic infection:
  • Use good oral hygiene.
  • Routinely brush and floss teeth and gums, and get regular checkups.
  • Cuts or lesions should be cleaned thoroughly and watched for signs of infection. A physician should be contacted if a cut or lesion is not healing well or looks infected.
Although physicians and dentists are aware that people who are susceptible to bacterial endocarditis should be given antibiotics prophylactically prior to procedures, it is a good idea to inform or remind your doctor or dentist of congenital heart disease before any dental, surgical, or special procedures is done.
Noncardiac surgery
All children and adults with heart defects must take extra precautions when undergoing a noncardiac surgical procedure. A relatively simple surgery like an appendectomy can be life-threatening for an individual with a complex congenital heart defect like Eisenmenger's syndrome because the hearts and lungs may respond unpredictably to anesthesia or the stress of surgery. Noncardiac surgeries may need to be performed at a special center that has cardiac anesthesia and cardiologists familiar with the complications of congenital heart defects.
During pregnancy, a woman's body goes through many changes, which may bring on or worsen the symptoms of a heart defect. This is especially true in the second and third trimesters. The mother's condition also can put the fetus at risk for poor growth or other problems. Some commonly prescribed heart disease medications pose added risks to the fetus. For some types of heart disease, especially those associated with pulmonary hypertension or significant ventricular dysfunction, pregnancy creates significant risk for the mother. In such cases, doctors would advise the woman not to have a baby.
With proper care, however, most women with congenital heart disease can successfully bear children. Prior to conception, women need to seek medical advice to fully assess the potential risk to the mother and baby. Proper medical advice will require assembling a team including the congenital cardiologist, a high-risk obstetrician, and possibly a local cardiologist and a genetic counselor as well. The multidisciplinary team will determine the best way to manage the pregnancy, the best way to deliver the baby, and whether there's need for special postpartum care for the mother.
In many cases, the team may recommend that the pregnancy be monitored by a high-risk obstetrician, as well as a cardiologist familiar with her condition.
In general, the risk of a fetus developing a heart abnormality is about 6 percent when the mother has congenital heart disease. However, each situation is unique and is largely dependent upon the type of heart defect in the mother, father or siblings. An accurate determination of the risk to the developing fetus may require genetic counseling.
Birth control
Women with congenital heart defects need to consult their primary care physician, gynecologist, and cardiologist before choosing a birth control agent. Intrauterine devices may be associated with an increased risk of endocarditis. Most women with congenital heart disease can use most of the effective methods of birth control safely. However, women with pulmonary hypertension or complex congenital heart disease, such as Eisenmenger's syndrome, should not use oral contraceptives or other methods of birth control that contain estrogen, because of increased risk of blood clots.
Associated conditions
Many people with congenital heart disease are at risk for pulmonary hypertension and heart arrhythmias.
Pulmonary hypertension is a debilitating and often fatal condition in which the blood pressure is elevated in the arteries that supply the lungs. Individuals with congenital heart disease that causes excessive blood flow through the pulmonary blood vessels are susceptible to developing pulmonary hypertension. For example, people with Eisenmenger's syndrome and mitral valve stenosis are at increased risk for developing pulmonary hypertension.
The early symptoms are fatigue and shortness of breath that become worse over time. As the condition progresses, chest pain, blueness of the skin, fainting, swelling of the legs, bulging of the neck veins, loss of appetite, and abdominal pain may develop. It is important to diagnose and treat this condition early on, before irreversible damage to the lungs occurs.
Some people diagnosed with pulmonary hypertension take vasodilators, medications that dilate the blood vessels, to control symptoms. The most effective of these, epoprostenol, is also the most complicated to administer, requiring continuous injection into a vein. In these cases, a permanent venous catheter is inserted, and the patient wears a small pump 24 hours a day. Other vasodilators can be taken orally in pill form or via a specialized inhaler. The blood thinner warfarin also can increase the life span in some people with pulmonary hypertension.
Arrhythmias, abnormal changes in the rhythm of the heartbeat, are not unusual in individuals with congenital heart disease. Arrhythmias may involve the heart beating too fast, too slow, skipping a beat, or adding an extra beat periodically. The symptoms of arrhythmias vary with severity from negligible to sudden death. Most frequently, arrhythmias involve sudden shortness of breath, lightheadedness, or fainting.
Arrhythmias can be lethal; any individual with congenital heart disease who becomes acutely short of breath needs to get an EKG immediately to identify any arrhythmias.
Exercise and congenital heart disease
Congenital cardiologists prescribe regular aerobic exercise for most people with congenital heart disease. Aerobic exercise strengthens the heart and circulation and improves overall physical fitness and emotional well-being. When exercising, people with congenital heart disease should always follow a physician's guidelines.
Many parents of children with congenital heart defects worry about the risks of rough play and vigorous activity on their child's heart. However, most children can engage in normal or near-normal activity levels. The parents may need to discuss their concerns with the pediatric cardiologist, who can advise parents on which activities are safe for a child. In certain instances, parents may need to encourage a child to pursue other interests, instead of focusing on the child's limitations. Although every circumstance is unique, most children with congenital heart defects grow up to lead healthy, productive lives.
Emotional well-being
Individuals with congenital heart disease face tough health issues throughout their lives. Often, they are coping with a lot of uncertainty about how their disease might progress. Family planning can be a critical issue for people with complex defects so as to avoid potentially life-threatening situations in terms of birth control and/or pregnancy. Planning for good health insurance so as to maintain access to one's doctors can be an added layer of stress.
Many of these issues arise during the transition from childhood to adulthood. Learning about one's condition is fundamental in order for preteens and young adults to make wise decisions that ultimately affect their adult lives. Selecting a career that is compatible with their condition and maintains lifelong medical insurance can help alleviate some stress. Proximity to specialized medical centers may need to be a factor when considering where to live.
Despite careful planning, feelings of anxiety and even depression are common among people coping with chronic health conditions, such as congenital heart disease. So if you think you may need help, seek it out. The Adult Congenital Heart Association is a nonprofit organization that seeks to improve the quality of life and extend the lives of adults with congenital heart defects. This national organization serves and supports more than 1 million adults with congenital defects, their families, and the medical community.
Last reviewed on 2/11/2009

Lost in transition: The looming epidemic of grown-up congenital heart disease

APRIL 12, 2011 Lisa Nainggolan
London, UK - Born with tetralogy of Fallot, the most common form of cyanotic congenital heart disease, Polly Logan-Banks had surgery at the age of two and yearly checkups at London's Great Ormond Street Hospital until she was 18. Now 26, she says other than "the scar" and her constant breathlessness, she "never really thought about her heart disease much further."
Between the ages of 18 and 25, Polly saw no one about her congenital HD—something absolutely typical of nearly all congenital HD patients, the vast majority of whom are lost to follow-up once they become young adults. As such, Polly and millions like her represent a new and growing phenomenon—young men and women who, a generation or two ago, may never have made it to their twenties. Had she not contracted endocarditis at the dentist last year—a cloud that turned out to have a silver lining—she would still be struggling with day-to-day activities, unaware of the fact that another operation could improve her symptoms and extend her life expectancy.
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.
We have talked about congenital heart disease as a pediatric disease that we can fix, and there has been the perception that we have cured it, but we haven't.
Also, the heart disease is not the only problem these adolescents face: in common with other kids who have chronic, lifelong diseases, they need advice over and above that which a normal teenager requires: on psychological issues, healthy behaviors, relationships, education, and career choices. In addition, topics such as genetic testing,contraception, pregnancy, and birth are especially important for young women with congenital heart disease. Other key topics include obtaining health insurance, a particular problem in the US and other countries with no state-funded healthcare, and life insurance, as well as making sure patients have realistic expectations about their life expectancy.
Dr Mike Gatzoulis
Dr Mike Gatzoulis
Dr Mike Gatzoulis (Royal Brompton Hospital, London, UK), who heads up what was one of the first grown-up congenital heart disease (GUCH) units anywhere in the world, established by Dr Jane Somerville (London, UK)—and which ironically is facing closure as a result of British healthcare cuts—told heartwire he believes his own profession has contributed to this problem.
"We have talked about congenital heart disease as a pediatric disease that we can fix, and there has been the perception that we have cured it, but we haven't, and this is a fundamental problem. Also, we haven't put what is the most common inborn defect into the main curriculum in medical school: it's a foreign subject, so a GP won't know very much about it," he says.
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.
Dr Barbara Mulder
Dr Barbara Mulder
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.
"To my mind, we should be talking about the process of transition at the moment of diagnosis, before birth," Gatzoulis says. "So you say to the parents, 'There are a lot of things that can help, but this is a lifelong disease and it's all about being proactive about health,'—that's when transition should start. I think most parents value this information: it tells them that a child may need an operation or two but that the child will live a long life and a fulfilled life. Although it's a shock to the system, at the same time it's a positive outlook."
Cardiology organizations, too, are recognizing that this problem needs to be addressed: the European Society of Cardiology issued new guidelines for the management of grown-up congenital heart disease last summer [4], and the American Heart Association issued a scientific statement, just last month, on best practices in managing the transition to adulthood for adolescents with congenital HD [5].

Looming epidemic: Adults with congenital HD outnumber kids
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.
Dr Peter Varga
Dr Peter Varga
"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."
Dr Gary Webb (Children's Hospital, Cincinnati, OH), who is considered by many as the one of the "fathers" of ACHD, now works in the US but spent his formative years at the world-renowned Toronto Congenital Cardiac Center for Adults. He is perfectly placed to contrast his recent experiences in the US with his years in the Canadian system.
Dr Gary Webb
Dr Gary Webb
"In Canada, pediatric hospitals will not admit a patient over 18, so when a kid hits this age, he or she must be transferred to adult care; this is seen as normal," Webb explained toheartwire"We have 20+ years of having a national set of ACHD clinics that collaborate with each other, and there are 15, all located at Canadian medical schools. People are expected to travel large distances, and they will often have regional coordination."
Care in the US for ACHD lags way behind that of Canada, says Webb, although he does point out that the magnitude of the problem is much greater in America, with 10 times the number of people affected there than in his home country. "One major difference is that, in America, pediatricians tend to hang on to their patients much longer than they do in Canada because there is no requirement for them to switch to adult care. The kids tend to get lost in that process because many of them just don't want to go to a pediatrician once they become adults," he says. And although there are some very good ACHD centers in the US—such as at Emory University, Atlanta, GA; the Mayo Clinic, Rochester, MN; the Children's Hospital Boston, MA; and Ohio State University, Columbus—"the US as a whole is poorly served in general," Webb observes.
90% are either receiving no care or receiving care in a very haphazard way in clinics . . . that are woefully unfamiliar with these very complex issues.
Varga, who runs a medium-sized ACHD clinic in Chicago, agrees: "There are around 70 adult congenital HD centers in the US, but maybe a quarter of those see a very large number of patients—about 600 to 1000 per year. Then there are programs like ours, which get 200 to 250 visits a year, and a smattering of smaller programs. But these existing resources cover only around 10% of adults with congenital HD. Costs are staggering, and we are not well prepared."
Another problem in the US is that ACHD clinics are not distributed equally, in geographic terms. "Much of the Midwestern part of the country has essentially no dedicated center addressing this need," says Varga, and Webb adds that many of the large urban areas are not well served either. "Chicago has a huge population and has very little in the way of ACHD care, and the same applies to Los Angeles and New York City," he observes. Varga agrees; even though his own institution and a couple of other medium-sized ACHD programs are based in Chicago, "clearly we don't meet the capacity," he says.
Varga says proper, organized care for adults with congenital HD "would require a kind of centralized planning that Americans are somewhat allergic to. In contrast, in some European countries and in Canada, there has been a very deliberate geographic distribution of these centers."

Should ACHD become a subspecialty?
In some countries, such as the US, there is talk of developing a certification in the subspecialty of ACHD.
Webb isn't too concerned about pieces of paper, however: "I think it's important to have highly qualified people looking after these patients, and how they got to be highly qualified is more important to me than what exams they've passed," he says. "When I was in Toronto, we used to train a lot of ACHD fellows and they would come from all over the world. We would say, "Okay, what are you going to do once you've finished your training here?" and they would say what their primary role was going to be—echo, or the cath lab, or EP—and we would train them with an eye to developing the skills they needed to go back and succeed in their new careers, rather than have a cookie cutter where all the cookies look the same."
It's important to have highly qualified people looking after these patients and how they got to be highly qualified is more important to me than what exams they've passed.
He stresses that the care of these patients requires people with different backgrounds and different interests. "To start with, you need excellent ACHD cardiology and echocardiography, and then you build from there, depending on resources and responsibilities, as you move along. Hopefully, you end up with a big team to handle all the various types of situations that arise: pregnancy management, genetics counseling, heart failure and rhythm problems, and devices and surgery and so on." One thing Webb has grown to admire in his new institution in Cincinnati is the use of nurse practitioners (NPs), "who play a much larger role in adult congenital HD care in the US than they do in Canada," he says.
And in the US, because there are very few mature ACHD clinics, "there are not a lot of jobs out there for doctors, which in turn discourages people from training in the area," Webb observes. "Moreover, there is no funding to train people even if one wanted to set up an ACHD training program."
Through the patient-advocacy group Adult Congenital Heart Association (AHCA), he has helped to establish and lead a "Vision 2020" process, in which "we began to look at what it would take to make high-quality ACHD care available throughout the US by the year 2020," but "this has stalled for the past year while ACHA tried to raise enough funds to get it going again," he laments.

"Business drives everything in the US"
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."

The Netherlands leads the way but is "unique in the world"
Mulder, who has been instrumental in the work done in the Netherlands, estimates that of around 30 000 patients in her country with congenital HD, only around a quarter to a third are lost to follow-up. The trick to catching these people, she says, is not to let them go in the first place. "At 18, most children finish school and are moving out of home and going to live on their own. They like to forget their medical past and try to escape. So it is important to immediately make a follow-up appointment, not leave it open, and to have a letter from the pediatric cardiologist."
The Dutch have a national working group on ACHD, and 13 000 of the patients there are tracked in a nationwide database, called CONCOR.
"The folks in the Netherlands have done a great job," says Webb. "They have been very collaborative going back 15 or 20 years and have worked together much better than people anywhere else in the world. In addition, they set up a national registry and have published extensively on this. They should be congratulated for all that they have done, but unfortunately they are unique in the world."
The folks in the Netherlands have done a great job but, unfortunately, they are unique in the world.
There are also some reasonably well-organized systems of adult care for congenital heart disease in Europe outside of the Netherlands, in Belgium, Germany, Sweden, and Switzerland, as well as the UK, says Mulder.
And there has been some progress in the more developed nations in Asia, she notes. "Japan has made a start trying to organize adult settings, and there are fledgling attempts to develop such programs in China and Indonesia." Australia "is more or less organized," and there are some centers in urban areas in the more developed countries in South America, she notes. But in most developing countries, the struggle is still centered on how to improve surgery rates for children with congenital heart disease. "If they are not operated on, they won't survive, so trying to focus on adult care in these countries is premature," she points out.
Both Mulder and Gatzoulis believe the key to good adult care of congenital HD is to have both pediatric and adult facilities on the same site. In the Netherlands, for example, there are four large tertiary referral centers for adults with congenital HD, Mulder explains. While the children and adults are seen in different units, they are usually within the same hospital, "and sometimes, for example in our institution, they see the same specialist but in a different department," she notes.
Gatzoulis—who did a fellowship in adult congenital heart disease under the direction of Webb in Toronto—says: "The model we have here at the Royal Brompton is excellent for transition: we have the whole of congenital HD on a single site: fetal cardiology, pediatric cardiology, and adult congenital HD or GUCH services, plus the institution is heart and lung," so issues such as pulmonary hypertension can be dealt with successfully, he explains. "We integrate with our pediatric colleagues on a daily basis; they come to our meetings and we go to theirs, so there is constant interaction and feedback, and there have been changes in terms of techniques: how early we operate and how we operate to prevent, as much as we can, long-term problems."
Webb agrees. In Cincinnati he is based at the Children's Hospital, which he believes "is the best place to have an ACHD clinic in the American environment." But his institution and Emory University are "to my knowledge, the only two institutions in the US that have been willing to make this commitment to transfer complex ACHD patients to adult care at age 18—something I see as central to the successful growth of the field." A recent study at Cincinnati Children's and one out of Belgium showed that the loss to care of complex patients has been very low, he says, and both of these occurred in places where the ACHD clinic was in the same building as the pediatric cardiology clinics, he notes. And although he acknowledges that this setup is not the norm in Canada, he says that "there is a fixed age of transfer to adult care at age 18 there, so this helps everybody behave in a reasonably consistent fashion."
Back in London, Gatzoulis and colleagues also run "transition clinics" four times a year. "Once patients approach the transition age, which we are flexible about, but in their teens usually, we will see them at least once with our pediatric colleagues and we also have a transition nurse," he explains. And while Polly missed out on such a clinic, she is appreciative of the fact that she was found and is embracing her future with renewed vigor. "When I had my first operation, I was two, and I was in a Great Ormond Street textbook: there was a chapter called 'Polly,' " she recalls. Now in this heartwire article, she feels she has come full circle: "My heart condition is making me 'world-renowned,' among doctors at least."

The patient's story: Polly
London, UK - Polly's brush with endocarditis in 2010 left her hospitalized but brought her back on the radar of congenital HD specialists. In March 2011, five days after undergoing open-heart surgery, which involved a tissue valve being implanted to reduce pulmonary regurgitation following the leaking of her own valve (originally operated on when she was two), Polly was bruised and suffering from a chest infection but happy to talk about what it meant to have been "found" again. Not only is her latest operation expected to improve her symptoms and life expectancy, she also received genetic testing, through which she discovered she has no greater risk of having a child with congenital heart disease than anyone else in the general population, something she describes as a "massive relief."
Polly Logan-Banks
Polly Logan-Banks
Polly says that while growing up, she was never really aware that she had heart disease. Both her parents died when she was young, and she was raised by her grandparents, who "tried their best" but didn't really grasp the nature of her condition and were unable to attend appointments with her. "I don't think it was impressed upon me very much how much of my unfitness was due to the heart condition, so all the time I was blaming myself for being unfit. And although she had regular annual checkups at Great Ormond Street Hospital in London, Polly says she does not recall ever receiving any advice on exercise, career choice, relationships, contraception—which, she says, "I didn't realize was an issue"—or any of the subjects that experts now agree should be addressed during adolescence. And although Polly's circumstances were more tragic than most, Gatzoulis says Polly's experiences in terms of the lack of advice she received are "typical."
Normal heart and tetralogy of Fallot/©Legger/ [Click on the image for a larger view]
At the age of 16, her grandfather died and at 18, Polly stopped attending the clinic. "I had a big 'what-the-hell' phase in my teen years," she says. "I'd had heart surgery and with my parents dying quite young as well, between the two I just kind of figured my life expectancy was going to be about 40, so I might as well just go out and have a good time. . . . Now I've had the new surgery, and they say, 'You might need to have more surgery when you are 50,' and I'm like, 'That means I'm going to get to 50—that's good, that's progress!' Had it not been for the endocarditis, I'd still be wandering around, out of breath."
During the time that Polly was lost to cardiology follow-up, she did see primary-care physicians, but she says that they never referred to her congenital heart disease. Asked whether her condition has affected her socially, she hesitates before answering. "Maybe it has affected me in terms of friendships, because I was so much slower than anyone else, so whenever we would go anywhere, it was always me lagging behind." And she has had "only one real long-term relationship, and he had Crohn's disease, so it was quite useful, because we both knew there could be medical problems for both of us." Polly works in a sedentary computing role, so her physical condition hasn't really affected her ability to work, she says.
Over the next six to 12 months, the size of Polly's right heart should significantly shrink, and this should markedly improve her symptoms, says Gatzoulis. Asked how she views her future, Polly says: "It would be so awesome to have more breath. Hills and stairs have been my nemesis for many a year."

Training cardiologists in ACHD care
All of the doctors who spoke with heartwire broadly agree that complex congenital HD patients, if they are stable, should be assessed once a year when they reach adulthood. "You need to have a look at the heart and the area of surgery and obtain a complete image of the great vessels," says Mulder, adding that, in terms of the long-term complications to expect, there are particular defects associated with specific types of congenital HD. For example, there are "a large spectrum of arrhythmias" that can occur, which can be treated with medication, ablation, or device implantation.
Webb explains: "There are a number of different types of tests that are done, with differing frequencies. Typically, patients have an ECG, and other tests include chest X-ray, echocardiogram, exercise test, MRI, a panel of blood tests, and a Holter monitor, among others. In Toronto, we built a computerized template so that every patient with a certain diagnosis would have a menu of tests that could then be reviewed with the patient at each visit and modified as appropriate."
For those whose disease is not severe enough to warrant continual assessment at an adult congenital HD unit, Mulder says these patients should at the very least see a general cardiologist periodically. This will become even more important in the future, as more and more congenital HD patients live longer and longer, and the problem of acquired heart disease is added to the mix.
Without a doubt, general cardiologists will need to be better educated on the subject of congenital heart disease in years to come, say the experts, but they admit this is no easy task. "Historically, the main way to learn about congenital heart disease was to see these patients, typically in the outpatient setting, during one's training," says Webb. "In Canada, there has been the opportunity for people to be exposed to congenital HD patients for the past 20 years, but in the US, few cardiology trainees see congenital HD patients because the clinics have not been organized and care has been dispersed.
"In my opinion, future learning will be over the internet," says Webb, who is holding a large ACHD course in Cincinnati in June in which seven successful teams from North America and Europe will present a total of 63 cases with a focus on learning; it will be posted online. "That said, cardiologists will only be willing to allocate so much time to this type of education," he observes. "Those of us working in the field will need to partner with those folks to identify the most effective way for them to meet their needs given the limited time available."