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Approximately 8 per thousand children are born with a defect in the anatomy of the heart or great vessels and around one half of these will require cardiac surgery or a catheter intervention. While many defects can be treated with a single procedure a number of patients will require a series of procedures over their lifetime. It is the principal job of the paediatric cardiologist to make the correct diagnosis, plan the treatment strategy and follow up of patients after surgery or intervention. In many cases follow up has to be lifelong. Drugs may have some place to ameliorate the effects of significant heart defects in the short term but usually improvement only takes place after definitive treatment. The key to successful outcomes is a close working relationship between cardiologist and surgeons together with other essential members of the team including intensivists, anaesthetists, nurses and allied health professionals.
Over the years, the growth of technology has had a huge impact on the role of the paediatric cardiologist although it is essential to maintain the centrality of the doctor-patient relationship and clinical skills. Major improvements in echocardiography and more recently CMR and CT have sharpened diagnostic precision and in so doing enhanced decision making. In particular, it is worth highlighting the advances in fetal echocardiography which allow accurate antenatal diagnosis so that families and clinical teams are prepared for the possibility of cardiac surgery soon after the child is born. Currently 50% of significant congenital heart disease is detected antenatally in the UK and this figure should continue to rise.
Catheter based technology has allowed the cardiologist to treat a number of defects using a non surgical approach. It has the principal benefits of avoiding surgical scars and allowing faster recovery. While it is unlikely that catheter intervention will ever replace the need for surgery, it does take away some of the demand on the surgeon and allows a strategy to reduce the number of surgical interventions over a lifetime for some patients. More recently the development of hybrid procedures, when catheter and surgical approaches take place at the same time, has provided innovative solutions to some very challenging clinical situations Outcomes for surgery and catheter intervention are audited in the publicly available NICOR (National Institute for Cardiovascular Outcomes) website.
As well as congenital structural cardiac defects, children can also be born with the tendency to develop heart rhythm disturbances due to defects in the electrical conducting system of the heart or the ion transport channels in the cell membrane. Advances include the use of catheter ablation and increasingly sophisticated pacemaker technology in terms of device size, rate responsiveness and ability to terminate arrhythmias. Ion channel defects are due to faulty genes as are many types of cardiomyopathy which means that this whole area interfaces with cardiac genetics and the increasing role of genetic testing in patient management.
With the above technological changes it becomes inevitable that Paediatric Cardiologists must specialise in a particular area to develop and maintain their skills. The exponential growth in knowledge of how to manage heart disease in children makes it more difficult for an individual to be an expert across all areas and also drives specialisation. Information of many of these areas of special interest can be found elsewhere on other pages.
Unlike heart disease in adults most children with heart disease are born with a problem as opposed to acquiring one. However there are some forms of acquired heart disease the Paediatric Cardiologist must be aware of. Worldwide the biggest acquired challenge is rheumatic heart disease with a prevalence of around 2% in most developing countries and which is due to an abnormal immune reaction to streptococcus viridans throat infection. This is thankfully now very rare in the UK where the main acquired cardiac conditions encountered in children are viral induced myocarditis, Kawasaki disease (which can involve the coronary arteries) and very rarely homozygous familial hypercholesterolaemia.
Two further areas of very specialised care that should be mentioned are cardiac transplantation and pulmonary hypertension. Both are now looked after by specialists focusing in these particular areas. Transplantation is linked with advanced heart failure management including the use of mechanical circulatory support and this only takes place in 2 UK centres. Pulmonary hypertension which is due is progressive resistance to blood flow through the lungs was once difficult to treat. However the development of new drugs and focusing of care into one national UK centre with a hub and spoke model of shared care has brought benefits to this patient group.
Finally the improvements in the outcome for congenital heart disease over the past 50 years mean that there are now more adults than children living with congenital heart disease. This has led to the development of the specialty of Adult Congenital Heart Disease which has an equal emphasis to Paediatric Cardiology within the BCCA and is covered on another page.
[Content provided by Dr Alan Magee, Paediatric Cardiologist, Southampton University Hospital - last updated 8 November 2016]
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