Hypoplastic left heart syndrome (HLHS) is the most severe form of congenital heart defect (CHD). The first successful intervention for it was undertaken by Norwood in 1983. Since then, there have been much development in the pre, intra, and postoperative treatment option in staged palliative surgical procedures. Early diagnostic management, prenatal interventions, innovative diagnostic methods, constantly modified surgical techniques, and hybridization contribute to a significant progress in treatment options. This will allow for defining an optimal strategy of improving survival and quality of life in HLHS patients. The development of intervention cardiology makes possible the stepwise treatment of the defect with one operation only. The first and third stage may be done by hybrid or interventional methods, then only the second stage of treatment needs to be done surgically. The world experience and all the available literature says that the 1st-stage procedure could be done now safely either directly or with a bridge to Norwood followed by the stage 2 with a Glen or Hemi-Fontan and followed by a Fontan down the lane surgically.

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