Hypoplastic left heart syndrome (HLHS) is a serious type of congenital heart disease where the left side of the heart fails to develop properly. It affects between three and five babies out of every 10,000 born. These babies are unable to pump blood around their bodies properly. Soon after birth they become pale and breathless and need immediate treatment to help them breathe. The only treatment for HLHS is to have a series of operations on the heart, often beginning within a few days of birth. Although surgery cannot cure HLHS, it can improve heart function and allow a child to live a relatively normal life. However, the operations are complex and even when successful, children often have ongoing health problems. At the moment, it is difficult for surgeons to decide exactly which surgical technique is best for each child. Information is limited because the condition is rare, and therefore it is not possible to study a large enough number of cases to reach concluding evidence. There is a need for more accurate and it has not, so far, been possible to analysise accurately of the clinical data and outcomes of the different procedures in different HLHS cases. If detailed information on the outcome of previous HLHS operations were available, this could make planning for new cases easier and give the best possible chance of success.
The research project
We plan to develop new analysis tools based on advanced medical imaging and computerized models of the heart, and to investigate if we could improve the surgical decision making process in HLHS. We are building computerized 3D models of each baby’s heart and major blood vessels so we can measure and compare the anatomy and function of the hearts in great detail. One hundred and fifty cases from two international centers in London and Boston, USA, are being analysed, and we are working to make the new tools available to any clinical center. It is hoped that this analysis will help heart specialists and surgeons to understand the impact of the different surgical approaches, and assist them in making the best plan for each case as it arises. Choosing the best treatment for each child should improve their chances of survival and quality of life as they grow and develop.
Further information: Action Medical Research