Surgeon looking at digital heart on a screen

The Newcastle Freeman Hospital department of Paediatric and Adult Congenital cardiology serves a UK region of just under 3 million people (5% of the total UK population). All congenital abnormalities are recorded on the region’s NORCAS (Northern Congenital Abnormality Survey) database and details of patients and outcomes are held on the departmental database. Access to this combination of information has led to several studies looking at the incidence and prevalence of congenital heart disease as well as analysis of risk factors including association with other congenital abnormalities.

A natural extension of this research was to use the data to predict the likely numbers of patients surviving to adulthood, and the degree of care they require as adults, and project this information onto the whole UK population. This 2001 study predicted 1600 patients per year transitioning from paediatric cardiology clinics to Grown Up Congenital Heart (GUCH) clinics across the UK. This data is still widely used to demonstrate the need for an increase in GUCH services and to illustrate the need for continued follow up of GUCH patients. More recently, closer examination of the single ventricle sub group has been undertaken. This clearly defines the 2013 UK single ventricle population at around 1040 patients and predicts growth in this population by 60% over the next decade. Also highlighted is the prediction that the proportion of patients with symptoms is likely to stay the same, meaning that the total number of symptomatic patients will increase with obvious implications for access to other services in particular transplant.

As the largest congenital heart disease transplant centre in the UK we continue to pay close attention to survival following heart transplant. In 2010 we demonstrated improvement in survival rates for GUCH heart transplants from 50% at five years in 1988-2001 to nearly 80% at five years at the most recent analysis. Although this improvement is a significant step forward for GUCH patients, particularly as we have shown the increase is due to improvement in patient management (as opposed to offering transplant to lower risk patients), there is clearly still work to be carried out in this area.

The next challenge that we see in cardiac transplant is to further develop our understanding of the risk factors for adverse reactions after heart transplant. Knowledge of these will enable to better predict when patients should be listed for transplant as well as how we can modify the risk factors to improve the outcome in individual patients. To this end we are hoping to publish data on transplant for Fontan patients. The key findings will be the importance of Fontan associated liver disease as a risk factor for transplant following the Fontan procedure, as well as that in some patients this risk can be lowered with proactive management while on the waiting list.

Although heart transplant is an extremely important part of advanced heart failure management in GUCH patients there are many patients assessed who are not suitable for heart transplant for a variety of reasons. Other patients, despite being suitable for transplant are simply too unwell to wait for a heart to become available. A long-term ventricular assist device (VAD), which is an implantable artificial heart pump, has been developed for patients with acquired heart disease, namely cardiomyopathy, often secondary to ischaemic heart disease (figure 1). The development of the Freeman’s VAD programme has produced much research in this area and there has been a particular focus over the last few years on the management of these patients at home. These pumps are ideally suited to certain GUCH patients’ circulations if heart failure develops, particularly those with a systemic right ventricle. We have published the largest worldwide experience with VAD for GUCH patients with a systemic right ventricle and now consider that offering such devices should be a standard part of management of heart failure in certain GUCH patients (figure 2). One of our current patients is so satisfied with the VAD that he has asked to be taken off the transplant list preferring to continue with the VAD!

Outside the field of heart failure we have had an increasing number of patients presenting with heart rhythm problems in association with congenital heart disease. Three areas we are currently looking at are atrial arrhythmias in the context of device closure of atrial septal defects; arrhythmia following atrial switch (Senning and Mustard procedure); and arrhythmia in repaired tetralogy of Fallot. We are hoping to publish our experience of treatment strategies for such patients later this year.

Areas not directly relating to the heart are also extremely important to GUCH patients. We have highlighted the issues of lack of advice or inappropriate advice given to women with GUCH with regards to contraception. We have shown similar problems with regard to problems accessing dental surveillance for GUCH patients, despite the importance of this as part of a strategy to reduce the incidence of endocarditis. Armed with this data we have brought dental surveillance services and contraception services into the GUCH clinic and these are now part of routine follow up appointments. This ensures that all can access these services as well as saving time for patients in terms of travel and, as a ‘one stop shop’, has proved extremely effective for patient care.

Outside the clinical areas there remains ongoing research at the Centre for Life with projects at present looking at the genetics of hypoplastic left heart syndrome (a rare congenital heart defect in which the left ventricle of the heart is severely underdeveloped) and the embryology of bicuspid aortic valves.

By David Crossland

Consultant Adult Congenital and Paediatric Cardiologist at Freeman Hospital, Newcastle upon Tyne