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Looking out for the ‘lost’ congenital heart patients

Beth Greenaway shares her personal thoughts on some of the reasons why so many congenital heart patients drop out of the system as adults, and why it is important for them to re-enter it

I don't remember my parents ever telling me that I had something wrong with my heart. I grew up thinking that there was nothing unusual about my regular cardiology appointments, or sick time spent away from school. I never felt that I missed out because I didn't have as much energy as my classmates, or was unable to participate fully in PE classes. I definitely excelled more in academic rather than sporting activities, but thankfully went to schools where athletic prowess was not important. I was lucky to have great parents who treated me like any other child, and allowed me to set my own physical limits. Aside from being smaller than my peers, and suffering from frequent colds and chest infections, there was little outwardly to suggest that I was not a normal healthy child.

Childhood surgery

In 1982, aged nine years, I had open heart surgery to repair my atrial septal defect and rebuild my narrowed pulmonary valve and artery. The transformation was miraculous, and I went almost overnight from being a 'sickly' child to one who put on a growth spurt and had loads of energy. I continued to have regular follow-up appointments, and understood that I would always need to take antibiotic prophylaxis before any dental treatment. Aside from a fading scar on my chest I had little to remind me of my cardiac history.

Teenage rebellion?

I had a strong desire to forget what had happened, and almost resented it when the subject was brought up by my parents, friends or medical professionals. For me things came to a head just after transition from pediatric to adult services, when I asked my new consultant if it was all right for me to start running to maintain fitness. His cautious answer, that it should be all right providing I didn't want to 'run mara-thons', was something that I didn't want to hear. This was certainly not my consultant's fault, it is just that advice an adult would recognize as sane and rational can still be seen as controlling and limiting by a teenager.

Psychologically, I suspect that my experiences as a child had more influence than I gave them credit for, and this response seemed yet again to differentiate me from everyone else. Like all teens I desperately wanted to ft in, and not be seen as some-one who had a disability.

Perhaps it is a marker of the stubborn nature of so many who have congenital heart defects, or simply my rather contrary nature, but I have been a recreational athlete ever since. Fuelled by being able to actually compete my ambitions grew, and I have since finished many running races, long distance triathlon, cycling races, and indeed several marathons!

Drifting away from follow-up

Shortly after this last appointment I went to university away from home, further complicating the scheduling of follow-up cardiology appointments. In common with so many, I viewed myself as 'cured' and indeed was never told anything different by my parents or consultants. I decided that attending further appointments was an unnecessary inconvenience, and simply got on with living my life.

After graduation I moved to Florida for eight years. Putting aside the bad habits adopted during my teen years I developed a very healthy life, eating well and exercising almost every day. Slowly, as I gained confidence in my body, I settled into an extremely active lifestyle.
I viewed myself as 'cured' and indeed was never told anything different by my parents or consultants
I became a competent amateur athlete doing endurance sports like triathlon. Itook advanced courses in sports physiology and became both a personal trainer and certified coach in a number of sports. Using the knowledge I gained, and always exercising with a heart rate monitor, I self-managed my heart condition. I felt that I had the situation under control and generally avoided all contact with the medical profession.

'All was not well'

It was not until sometime after I returned to the UK in 2002 that I started to realize that all was not well heart-wise. For several years I had experienced what I knew to be arrhythmias, but now found myself feeling increasingly tired and weary. I put this down to the stress of the move, buying a house, getting a job, and building a new life away from my friends. My race results also started to go down, something I was content to rationalize as increasing old age as I approached my 30th birthday!

Finally, in October 2002, after a particularly awful three days of sustained rhythm disturbances, I decided that I could bury my head in the sand no more. Having successfully avoided cardiologists for so long, I did finally see sense and paid a visit to my GP. Having berated me for not taking care of things before, she immediately referred me to the local hospital in Plymouth for tests, thus beginning the bumpy journey to where I am today.

The first cardiologist I saw was an electro-physiology specialist, who, having taken my medical history and sent me for an echocardiogram, made the wise decision to send me on to see the GUCH (Grown Up Congenital Heart) consultant. Imagine my embarrassment when this was the same doctor I had last seen more than 10 years previously, when I had taken such offence at his wise and considered words. Evidently, the intervening years had matured and mellowed me, as after being very scared of him as a teenager we now have a wonderful relationship.
my years of lost follow-up had allowed my condition to deteriorate
I could tell from the look on his face that all was not well. It took him very little time to diagnose severe pulmonary regurgitation with significant dilation of the right ventricle and pulmonary artery. Although not immediately life-threatening, it was obvious that my years of lost follow-up had allowed my condition to deteriorate. My symptoms would continue to get worse without treatment, leading to irreversible damage to my heart.

Further surgery

The news was a real shock, and I felt angry that my body had let me down in this way. My high levels of fitness and healthy life-style had suppressed and masked the symptoms for many years, but there was no longer any denying that I had a serious problem.

The difficulty for my consultants was that the lifespan of a bio prosthetic pulmonary valve is not infinite, owing to calcification and stenosis, so once replaced the clock is already ticking on further re-intervention.

It is a case of leaving things long enough that you minimize the need for frequent surgeries, but not so long that the patient is very unwell and permanent damage has occurred. There have been some recent innovations in percutaneous delivery of replacement valves via catheter, avoiding the need for repetitive and risky open heart surgery. Sadly, with my dilated pulmonary artery I was not a suitable candidate for the trial, but with luck any future operations should be able to be done this way.

I am unsure if it was the realization that I really was seriously ill, or if it was because my health continued to decline, but I found the many months of waiting very hard. I was increasingly exhausted, and struggled mentally with what I saw as vastly declining physical ability.

It was with a strange sense of relief that in August 2005 I finally had open heart surgery at the Bristol Royal Infirmary to replace my pulmonary valve. I can't speak highly enough of the standard of care that I received there. The reality of going into hospital as an adult, fully aware of the possible risks of surgery, and the perceptions of a naïve child are very different. Equally, in terms of how quickly I was encouraged to get out of bed and move around, the contrast between my stay in 1982 and that of 23 years later was startling.

I spent only six days in the hospital, and made a remarkably quick recovery. I believe that this was due in no small part to my previous healthy lifestyle, and prior training as an endurance athlete. I was keen to regain my fitness, and worked hard to exercise as much as my recovering body would allow. I was even feeling well enough to race in a small local triathlon only six weeks after surgery, something not recommended in any cardiac rehab manual!

Lessons to be learned

So what can be learned from my story? Like many teens that had spent their child-hood in hospital and doctors' offices, I stopped going as soon as I could. It is common for GUCH patients to fall out of the system during their teen and early adult years, as they transition from pediatric to adult care. Like me, many teens believe that if they are functional then they are indestructible, and just want to get on with their lives.

I am extremely lucky. Those years without follow-up probably made little difference to my outcome, but for other GUCH patients who have left the system, or are being seen by doctors not versed in their highly specialist needs, it is often far too late when serious problems start. Apparently stable patients can deteriorate very quickly without appropriate care.
Procedures which were once spoken of as 'fully curative' are now being shown to be only temporary repairs
We are learning that those who had successful surgery as children often go on to need further intervention later in life. Procedures which were once spoken of as 'fully curative' are now being shown to be only temporary repairs.

A surprisingly high percentage of GUCH patients experience arrhythmias caused by scar tissue, turbulent blood flow or dilated chambers. Having learned to live with an abnormal heart which sometimes 'acts up', it is easy to delay getting specialist care by rationalizing that these events are just par for the course.

Some countries estimate the percentage of adults with congenital heart disease lost to follow-up to be as high as 80%, meaning that there could be literally tens of thousands of such adults in the UK with what has been described as a 'ticking time bomb' in their chests. Without regular follow-up by a GUCH specialist, the prognosis can be poor.

The need for specialist care

The 2006 NHS commissioning guide for services for young people and adults with congenital heart disease, written with the assistance of the GUCH Patients Association (former name of The Somerville Foundation), now clearly states that all adults with congenital heart disease, whatever the level of complexity, should be seen by an expert from a specialist centre at least once, and receive a written care plan (DH Vascular Programme Team, 2006).

There are currently 13 expert GUCH centres in England recognised by The Somerville Foundation as being able to diagnose and treat GUCH patients.

The Somerville Foundation also provides advice and support to a growing number of patients and their families. Services include social gatherings, weekends away, an active online message board, freephone helpline, information leaflets, and government and public lobbying to raise awareness of GUCH issues.

Since my surgery, I have regained much of my previous vigour and passion for life. I will never be free of arrhythmias, and further intervention is inevitable with my homograft valve. However, I recognise how different the outcome could have been, and many GUCH patients are not so fortunate.

There has been a huge effort to create a number of specialist GUCH centres around the country, each staffed by a multi-disciplinary team of experts in diagnosing and treating GUCH patients. They are saving and prolonging lives every day, but can only help those who are in the system. It is vital to refer every 'lost' GUCH patient to one of these specialist centres, where they can all be evaluated and provided with an ongoing care plan. Doing so may well save their lives!

This article was first published in the British Journal of Cardiac Nursing - April 2008, Vol 3 No 4. Updated September 2012.

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