title: Hypoplastic left heart syndrome: a review.
abstract: BACKGROUND
  Hypoplastic left heart syndrome (HLHS) is an etiologically multifactorial congenital heart disease affecting one in 5,000 newborns. Thirty years ago there were no treatment options for this pathology and the natural course of the disease led to death, usually within the first weeks of life. Recently surgical palliative techniques have been developed allowing for a five-year survival in more than half the cases.
  
  
  METHODS
  We reviewed literature available on HLHS, specifically its anatomy, embryology and pathophysiology, and treatment. The Pubmed and ClinicalKey databases were searched using the key wordshypoplastic left heart syndrome, foetal aortic valvuloplasty, foetal septoplasty, Norwood procedure, bidirectional Glenn procedure, Fontan procedure, hybrid procedure.The relevant literature was reviewed and included in the article. We reported a case from Children's Clinical University Hospital, Riga, to illustrate treatment tactics in Latvia.
  
  
  RESULTS
  There are three possible directions for therapy in newborns with HLHS: orthotopic heart transplantation, staged surgical palliation and palliative non-surgical treatment orcomfort care.Another treatment mode - foetal therapy - has arisen. Staged palliation and full Fontan circulation is a temporary solution, however, the only means for survival until heart transplantation. Fifty to 70% of patients who have gone through all three stages of palliation live to the age of five years.
  
  
  CONCLUSIONS
  The superior mode of treatment is not yet clear and the management must be based on each individual case, the experience of each clinic, as well as the financial aspects and will of the patient's parents.
authors.affiliation: Riga Stradiņš University, Riga, Latvia.
authors.forename: Roberts
authors.initials: R
authors.lastname: Gobergs
authors.affiliation: Riga Stradiņš University, Riga, Latvia.
authors.forename: Elza
authors.initials: E
authors.lastname: Salputra
authors.affiliation: Riga Stradiņš University, Riga, Latvia.|Department of Cardiology and Cardio Surgery, Children's Clinical University Hospital, Riga, Latvia.
authors.forename: Ingūna
authors.initials: I
authors.lastname: Lubaua
keywords: Fontan procedure; Norwood procedure; balloon valvuloplasty; bidirectional Glenn procedure; hybrid procedure; hypoplastic left heart syndrome