Congenital heart disease – predicting the outcomes of heart surgery

Around one in every 5,000 babies is born with a serious heart defect called hypoplastic left heart syndrome (HLHS).[1] They will all need a series of life-saving operations during their first few years of life. But unfortunately, some will go on to develop serious complications later in childhood. Dr Adelaide de Vecchi of King’s College London is finding ways to identify which babies are most likely to experience some of these problems before they occur. Predicting the long-term outcomes of heart surgery should improve babies’ chances of living longer, healthier lives.

How are children’s lives affected now?

Babies who are born with HLHS are unable to pump blood around their bodies properly because the left side of their heart fails to develop properly. Without treatment, they will quickly become seriously ill.

“Soon after birth, they will become pale and breathless and need immediate treatment to help them breathe,” says Dr de Vecchi. “The only treatment is to have a series of operations on their tiny hearts, usually beginning within a few days of birth.”

The first operation involves reconstructing part of the baby’s aorta, the main vessel that carries blood to the body. While this life-saving surgery aims to improve blood flow, it alters the shape and function of the aorta – and unfortunately, many children will experience negative consequences as they grow up.

“By the age of six, many children will have a widening or stiffening of different parts of the aorta, putting them at greater risk of heart failure,” says Dr de Vecchi. “Sadly, these complications can severely affect a child’s life expectancy and long-term quality of life.”

How could this research help?

Using magnetic resonance imaging (MRI) scans to image both anatomy and blood flow in newborn babies and children with HLHS, the researchers will look for early indicators of problems occurring in the descending aorta – the part of this vessel that passes through the chest and abdomen.

“We hope to identify specific features that can predict the likelihood of a child experiencing widening or stiffening of the descending aorta before their sixth birthday,” says Dr de Vecchi. “By identifying those most at risk earlier, this would help enable the delivery of personalised care – giving each child the best possible chance of doing well in the longer term.”

In addition, a better understanding of the long-term outcomes of reconstruction of the aorta will also help to inform surgical techniques – improving the outlook for babies born with HLHS and their families in the future.

References

1. Khairy P et al. Univentricular heart. Circulation. 2007; 115(6): 800-12.

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