Paediatric Cardiology

Information for Healthcare Professionals

Introduction

Paediatric cardiology may be defined as the field of medicine dealing with the diagnosis and treatment of childrens’ heart conditions. This comprises both acquired, inherited and congenital heart disease (CHD). With technological developments and improvement in life expectancy, the role of the paediatric cardiologist has extended in both directions giving rise to specialists in fetal cardiology and adult congenital heart disease.

Congenital Heart Disease (CHD)

CHD is the most prevalent congenital defect affecting approximately 5 -9 per 1,000 births. The spectrum of CHD ranges from minor defects (small holes in the atrial or ventricular septum, mild valvar lesions) which will not require treatment nor impact quality of life, through to very complex defects which are incompatible with a reasonable quality of life or life expectancy despite the most cutting-edge therapies.

From the 1970s, advances in cardiac imaging, medical treatments and surgical/ catheter intervention strategies have revolutionised the prognosis for babies with CHD. In the UK, 98% 30 day survival is documented following paediatric cardiac surgery (NCHDA 2024). Despite this success story many conditions remain challenging to treat effectively with roughly, 4 in 10,000 fetuses born with a functionally single ventricle circulation. These infants are not amenable to “corrective” surgery but instead may be candidates for a staged surgical palliation culminating in a Fontan circulation. The outlook for these babies is more guarded such that approximately 70% would be expected to survive through to adulthood.

Despite improving surgical and interventional results it is well evidenced that children and young people with CHD have different life experiences and face significant challenges throughout their life. Consequently psycho-social issues and neurodevelopmental outcomes are increasingly being recognised as a vital area of research and target for therapeutic interventions support. Due to the success of paediatric cardiology, there are now more adults than children with CHD.

Acquired Heart Disease in Childhood

In developed countries, most heart disease in childhood is congenital, inherited or due to arrhythmia. However, there are important acquired heart diseases such as viral induced myocarditis and Kawasaki disease (which can involve the coronary arteries) that are the responsibility of the paediatric cardiologist. Worldwide rheumatic heart disease remains a significant challenge, with a prevalence of around 2% in developing countries. More recently the COVID-19 pandemic caused a spike in acute cardiac complications in children (PIMS-TS) which fortunately only rarely has any long lasting cardiac effects. However, the impact of the COVID-19 pandemic on young people’s general well-being including symptoms warranting cardiac investigation has been significant.

Inherited Cardiac Conditions

More recently the sub-specialised area of Inherited Cardiac Conditions, concentrated primarily upon channelopathies and cardiomyopathies, has arisen which has been aided by rapid advances in genetic medicine. Our understanding of these conditions remains frustrating but has improved greatly over the past 20 years and is expected to continue to improve.

Configuration of the specialty in the UK and Ireland

Paediatric Cardiology requires the expertise of a multi-disciplinary team, the size and scope of this team increasing exponentially over the past 30 years, including many medical professionals (cardiologists/ surgeons/ anaesthetists/ intensive care physicians and radiologists) as well as specialist nurses, cardiac physiologists, perfusionists and other Allied Health Professionals to deliver the best care for patients. Whilst paediatric cardiology itself is a highly specialised field, the progress in diagnostic and therapeutic options has driven further subspecialty interest as highlighted in the working groups and senior training subspecialty areas – Fetal cardiology, Advanced echocardiography, Cardiac cross-sectional imaging, Interventional catheterisation, Electrophysiology, Heart failure and transplantation, Pulmonary hypertension and Inherited cardiac conditions.

Paediatricians with Expertise in Cardiology (PEC) have grown to meet the demand for specific cardiology evaluation and treatment delivered more locally. PECs also play a vital role in supporting Neonatal Intensive Care Units and the Paediatric Cardiology outreach service delivered across Level 3 hospitals.  

Congenital cardiac services in the UK and Ireland are configured in “Hub and Spoke” networks comprising Level 1,2 and 3 centres. Regional networks work with commissioners, providers and patients to ensure the delivery of safe and effective services across the patient pathway and help secure the best outcome for patients. Local congenital heart disease networks (link to CHD networks in patients section) provide a range of educational and networking opportunities for professionals and opportunities to get involved and help shape local care.

Paediatric Cardiac surgery and catheter interventions are performed at twelve Level 1 centres across the UK and Ireland. There are a further five Level 2 centres which deliver the full range of paediatric cardiology services apart from cardiac surgery/ catheter intervention. In a larger number of Level 3 centres care is delivered by Paediatricians with Expertise in Cardiology who are linked to a Level 1 +/- 2 centre. Due to the small number of patients requiring these highly specialised services, cardiac transplantation is centralised in two centres (Freeman and Great Ormond Street Hospital) and pulmonary hypertension in Great Ormond Street Hospital with extensive outreach.

Education and Training

Paediatric Cardiology has been a recognised specialty for more than 30 years. There is a well-structured training scheme with a highly developed governance structure, more information can be found here. The Specialty Advisory Committee for Paediatric Cardiology welcomes applicants from both general paediatric and adult cardiology training backgrounds. In the Republic of Ireland, Paediatric Cardiology has more recently received specialty recognition. Paediatric Cardiology training on the Island of Ireland is overseen by the Royal College of Physicians of Ireland.

Audit and outcomes

Cardiac surgery, cardiac catheterisation, electrophysiology procedures and antenatal detection rates, for each institution in the UK and Ireland, are comprehensively audited by National Congenital Heart Disease Audit (NCHDA) more information can be found here.

Key documents

Updated: Dr Brian McCrossan Nov 2024