Improving care for patients with congenital heart disease, from birth to adulthood

Each day in the UK around 13 babies are diagnosed with a congenital heart condition, which means a heart problem has developed before the baby is born.

When the BHF was founded in 1961, the majority of babies diagnosed with severe congenital heart disease did not make it past their first birthday. Today, 8 out of 10 survive to adulthood.

The cutting edge of surgery

In the 1970s, Magdi Yacoub (who later became BHF Professor of Cardiothoracic Surgery) developed a surgical technique to repair a defect in which two of a baby’s major blood vessels carrying blood away from the heart are the wrong way round (transposition of the great arteries). This life-saving operation, called the arterial switch, is now used by surgeons across the world and is successful in over 95 per cent of cases.

Throughout the 1970s and 1980s, BHF Professor Robert Anderson helped to improve surgical treatment of children with congenital heart disease by carefully mapping the anatomy of different heart defects and showing how these affected the heart’s electrical system that coordinates the heartbeat. This has enabled surgeons to avoid putting a stitch where it could disrupt an electrical circuit in the heart, making surgery safer.

Until two decades ago, replacement of faulty heart valves in children and adults required open heart surgery. In 2004, we funded research at Great Ormond Street Hospital to develop a quicker and less invasive technique to replace heart valves, using a tube inserted into a blood vessel in the top of the leg to reach the heart. Thousands of patients around the world, including children and adults with congenital heart disease, have now benefited from this procedure and have avoided open heart surgery.

Dealing with a common complication of congenital heart disease

Pulmonary hypertension is a potentially life-threatening condition caused by high blood pressure in the arteries of the lungs, causing damage to the right side of the heart. It can be a complication of some types of congenital heart disease.  

From the 1970s, BHF Professor Glennis Haworth and her team at the Institute of Child Health in London researched the development of pulmonary hypertension in children with congenital heart disease. She showed that damage to the blood vessel in the lung often occurs before birth, and may not recover even when the heart defect that caused the problem has been repaired with heart surgery. At the time, there was no adequate drug treatment and these children survived for a few years at most.

By 2002, the great strides in new treatments for pulmonary hypertension in adults offered a potential solution although one needed care in using drugs developed for adults in children. To take advantage of this, Professor Haworth founded the UK Pulmonary Hypertension Service for Children in 2002, which she led for many years. This clinical network, then the first of its kind in the world, helped to identify the best treatments for children with pulmonary hypertension, and still cares for children throughout the UK with the condition. Professor Haworth’s work has been instrumental in improving the survival of these children.

The survivors grow up

BHF-funded advances have helped to significantly reduce the number of children dying from congenital heart disease and improved survivors’ quality of life. But this has come with new challenges. It became clear that many survivors require lifelong care, as some are at risk of heart failure and sudden death from unexpected heart rhythm disturbances.

By the 1970s, surviving babies were growing into toddlers and school children and, by the 1980s, there were many more approaching adolescence. We funded work by Professor Jane Somerville at the Royal Brompton Hospital, who was one of the first people to recognise the importance of caring for the growing number of adult survivors of congenital heart disease, developing the idea of ‘GUCH’ — grown-up congenital heart disease – and pioneering GUCH care in the UK and Europe. The BHF funded Professor Somerville to create a follow-up system for survivors of congenital heart disease to identify which factors make a difference to their long-term survival.

Since then, we have funded research to find out how to best care for adults living with congenital heart disease. Even when the defect has been repaired with surgery in childhood, many still face an increased risk of heart failure or heart rhythm disturbances that can lead to sudden death. In 2011, we funded Dr Sonya Babu-Narayan’s work at Imperial College London to refine ways of correctly identifying which adults born with tetralogy of Fallot are potentially at increased risk of sudden death. Using cardiac MRI, Dr Babu-Narayan and colleagues worked out how heart scarring seen on imaging scans can be used to predict which adults with repaired tetralogy of Fallot need an implantable cardiac defibrillator (ICD) because they are at high risk of life-threatening heart rhythms.

Now there are more adults than children living with congenital heart disease, thanks to the successes of pioneering heart surgery and recognition of the special needs of GUCH patients.

First published 1st June 2021