Guidelines for the management of neonates and infants with hypoplastic left heart syndrome: EACTS and the AEPC Hypoplastic Left Heart Syndrome Guidelines Task Force

A widely cited description of hypoplastic left heart syndrome (HLHS) was provided by Lev in 1952, although references to this anatomical entity had appeared sporadically over the preceding 400 years. The syndrome was named in 1958 by Noonan and Nadas [1, 2]. Although specific figures for all of Europe are not readily available, the HLHS phenotype occurs in ∼0.016–0.036% of live births in Canada and the USA. Thus, each year ∼56–126 newborns in Canada and 640–1440 in the USA are affected [3–7]. Estimates for the incidence of HLHS (among all live births) in Germany, Croatia and Belgium are 0.016%, 0.017% and 0.009%, respectively [8]. The incidence is probably in transition to some extent, because accurate foetal diagnosis and termination of pregnancy have become increasingly prevalent in many countries. HLHS represents 2–9% of congenital heart disease (CHD) cases but accounts for 23% of neonatal deaths from congenital heart malformations [9–13]. Although it is not the most critical issue in child health worldwide, success with treatment of HLHS has become a new standard for assessment of the quality of care delivered by cardiac teams worldwide. Importantly, the technology and knowledge base developed for treatment of HLHS have had far reaching implications for dealing with other complex paediatric problems, both cardiac and non-cardiac.