Severe heart defects put children at 30 times greater risk of death before age of one

A child born with a severe heart defect is 30 times more likely to die before the age of one than the rest of the population, according to new research funded by the British Heart Foundation (BHF).

While children with a congenital condition are most likely to die in infancy, over 20 per cent of childhood deaths from severe heart defects take place after the child's first birthday. This shows a heart defect can leave children vulnerable for many years after surgery.

The research, published in the online journal PLOS ONE ⁽¹⁾, combined data from all of the paediatric cardiology centres in the UK giving a unique insight into severe forms of congenital heart disease, which can be life-threatening and require surgery in the first year of life. The comprehensive findings could inform care priorities and future research.

The researchers found that long-term survival and health outcomes are influenced by health events across the life course. They conclude that future improvements in survival and quality of life of adults with congenital heart disease are as reliant on optimising the health experience of these people at preschool- and school-age as successful early surgical repair.

Improvements in treatment mean that 90 per cent of the children born with heart defects, who reach their first birthday, survive beyond the age of 15. Despite these figures, the findings show how these conditions and their treatment can have long-term health impacts. The results suggest that the medical community should adopt, from birth, a "whole life strategy" to care for someone with a heart defect.

With thousands of children starting school for the first time this year, the BHF has launched Bag it. Beat it. to help raise vital funds for research so more children born with heart defects can make it to school. During Bag it. Beat it. the BHF is urging the public to donate bags full of their unwanted items to BHF shops to raise money for life-saving heart research.

This study is a unique collaborative effort and shows how individual centres can work together to share information. The study was funded by a Project Grant from the BHF and a Medical Research Council (MRC) Training Fellowship. It was led by researchers from the UCL Institute of Child Health in partnership with Great Ormond Street Hospital ⁽²⁾.

Dr Rachel Knowles, lead researcher, from the University College London (UCL) Institute of Child Health, said:

"We know that children born with congenital heart defects are living longer year on year so there may be an even better outlook for a child born today than in this study, which began in the 1990s.

"Studies that look at the longer term outcome for children with congenital heart disease are becoming ever more important for children, their families and their doctors. We need repeated long-term studies, which also raise awareness of the importance of the child's perspective and their quality of life."

Professor Peter Weissberg, Medical Director at the BHF, which funded the study, said:

"These important findings show that more and more children with congenital heart defects are surviving into adulthood, thanks to improved surgical techniques and early diagnosis. Both of which have been made possible by research.

"But the study also emphasises that those children who survive their first year with a heart defect are still at greater risk of death in subsequent years than those born without. The study shows that being born with congenital heart defects has lifelong consequences and that a whole life strategy for medical care is required from the outset. This will help ensure that more children and adults with heart defects have the greatest chance of living a long and healthy life."

This study could help to improve treatment and care for children like Calum Morris, aged eight from Bristol, who was born with Truncus arteriosus - a condition where the two main arteries leaving the heart are joined together as a single artery instead of two separate arteries. Calum had his first surgery when he was just two weeks old, followed by two more operations in the next few years of his life. Just before he turned seven, an infection led to him needing four further operations.

The teams compiled data from around 4,000 children with severe congenital heart defects born in the UK between 1993 and 1995 who were followed up with into adolescence. The aim was to investigate survival and quality of life for children with a range of congenital heart defects that required surgery in the first year of life.

The study team highlighted the importance of focussing on quality of life and health of children as they grow up and become adults. This is one of several papers about this group of children and taken together they provide rich insights into the experience of older children who have grown up with congenital heart disease.

The BHF is urging the public to get involved in Bag it. Beat it. this September by simply filling a bag with good quality clothes, shoes, books, handbags, DVDs, CDs, bric-a-brac and children's toys and taking it into their local BHF shop. Every bag donated will fund life-saving heart research, helping over 40,000 little heroes continue to fight congenital heart disease.

To find your local BHF shop or to book a FREE collection of your unwanted items visit: bagit.bhf.org.uk or call 0800 915 7000.