title: [Hypoplastic left heart syndrome (general review)].
abstract: The hypoplastic left heart syndrome (HLHS) is a critical congenital heart lesion with a small left ventricle in combination with stenosis or atresia of the aortic and the mitral valve. This heart disease is associated with a nearly 100% mortality at neonatal age. Until recently, this heart lesion was considered inoperable because of extremely unfavourable morphology. Introduction of the Norwood three-step reconstructive operation on one hand, and heart transplantation in neonates, on the other hand, improved the prognosis of patients with this complex heart lesion. In our country, however, this heart disease, if found during the first months of pregnancy, represents an indication for its termination, and neonates with HLHS are offered only symptomatic medical treatment. On basis of good experience from several most experienced cardiac centres we believe that it is necessary to reassess the statement considering HLHS as an inoperable disease. The main change from this aspect necessitates, however, a detailed analysis of the whole problem not only from the morphological, clinical and surgical point of view but also from philosophical, psychological, socio-economic and health care organisation aspects.
authors.affiliation: Dĕtské kardiocentrum, Fakultní nemocnice v Motole, Praha. tomas.tlaskal@lfmotol.cuni.cz
authors.forename: T
authors.initials: T
authors.lastname: Tláskal
authors.forename: V
authors.initials: V
authors.lastname: Povýsilová
authors.forename: D
authors.initials: D
authors.lastname: Vondrys
authors.forename: J
authors.initials: J
authors.lastname: Skovránek
mesh_terms: D006348:Cardiac Surgical Procedures; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000601:surgery*; D007231:Infant, Newborn; D011247:Pregnancy; D011296:Prenatal Diagnosis