What is hypoplastic left heart syndrome (HLHS)?

What is hypoplastic left heart syndrome (HLHS)?

Hypoplastic left heart syndrome (HLHS) is a group of birth defects in which left side of the heart is poorly developed. Left atrium, left ventricle and aorta can be poorly developed and the valves in between – the mitral and aortic valves – can be poorly developed or atretic (without an opening). Survival requires surgical repair, often complex and multiple procedures. One of the most popular operations is Norwood operation which utilises the right ventricle to pump blood into the whole of the body (systemic pump).

Cardiac transplantation in newborn period as the primary operation for HLHS is another option. Though cardiac transplantation is a seemingly better treatment for HLHS, it is limited by the availability of new born hearts. Moreover there is a chance for rejection with need for lifelong immunosuppressive treatment and limited survival of the transplanted heart, currently about 15 years.