New recommendations for patients with congenital heart disease

Nov 8 2013

ESC Working Group and Association publish first-time position paper

The diagnosis and treatments of congenital heart disease have improved so much that many babies now born with heart defects can still look forward to a long and fulfilling life. Just two generations ago, the majority of babies born with heart defects died before their first birthday, but today many of these conditions can be corrected. Indeed, the population of patients with congenital heart disease who reach adulthood - and the number of adults - is now expected to grow at a rate of 5% per year. However, residual lesions may persist such that some patients may still need lifelong care.

Many in this newly emerging patient group have been heavily protected from birth and encouraged towards a sedentary non-disruptive lifestyle. In fact, one study found that only one in five children with congenital heart disease had ever been given formal advice on physical activity. Yet the truth is that exercise is as beneficial for this group as it is for any other; but which physical activities and with what frequency and intensity?

New recommendations published today by the Working Group of Grown Up Congenital Heart Disease and the Section of Sports Cardiology of the EACPR emphasise that the majority of those with congenital heart disease will achieve "significant improvement of their exercise capacity as well as their psychological state". The key, says the report's first author Professor Werner Budts from the University Hospital Leuven, Belgium, is that the activity is appropriate for each individual.

"We hope that these recommendations will offer reassurance to patients and physicians alike, and encourage a physically active life style, so that patients with congenital heart disease can achieve the benefits of regular exercise at the lowest possible risk," said Professor Budts.

The recommendations - the first ever specifically for adolescents and young adults with congenital heart disease - cover a wide range of physical exercise, from everyday activity to participation in leisure time sports, with the recommendation of individualised exercise prescription based on five hemodynamic parameters: ventricular function, pulmonary arterial pressure, aortic diameter, arrhythmia, and arterial saturation. These five parameters provide a basis for evaluation and for a tailored approach to each individual.

Once these five parameters have been assessed, the programme of activity can be safely introduced with an intensity appropriate to each patient ("relative intensity"). It is for this reason, says Professor Budts, that a wide range of activities has been included in the recommendations, so that most patients with congenital heart disease can find a physically appropriate activity and can be positively encouraged. Some "high static" sports, for example, are included (such as water skiing and rowing) as are team games. Participation in team sports, in particular, are encouraged, where levels of similar physical fitness might be found.

But, warns Professor Budts, "patients should monitor their symptoms and heart rate to ensure they do not exceed the recommendations, at least for prolonged period of times, particularly for start-stop sports like football". He adds that those who feel that their fitness is lower than that of their team-mates should be supported "to either find an alternative team or sport".

"So the recommendations also offer physicians a decision platform to help keep patients within exercise limitations and ensure their sports are safe for them," says Professor Budts.

"However, activity advice for those with normal hemodynamic and electrophysiological status will not differ from the general population. Only for those with residual lesions will recommendation be more restrictive. In these cases the severity of some lesions causes an additional pressure or volume load on the heart and great vessels which could become unacceptable at certain intensities. Being more (but not too) restrictive could avoid further damage.

"Yet, for the majority of young adults with congenital heart disease we believe that patient-tailored advice will improve health behavior, influence positively their cardiovascular risk profile, and ultimately benefit outcome."