Despite approximately 50 years of progress in the management of com
plex congenital heart disease, hypoplastic left heart syndrome
(HLHS) has been one of the last cardiac malformations to yield to
effective surgical treat ment. The surgical therapies for
hypoplastic left heart syndrome have evolved by two quite disparate
routes. One approach has been the staged reconstruc tion operations
utilizing an initial operation pioneered by many but developed by
Dr. William Norwood and subsequently modified by many other
investigators. The first stage reconstruction operation has been
refined and standardized such that the majority of infants now
proceed to second and third stage reconstruc tive operations
culminating in a Pontan Kreutzer single ventricle physiology. An
alternative surgical approach has been the development of neonatal
and infant heart transplantation for HLHS. This approach also has
had a signifi cant impact on the treatment of HLHS and other
cardiac malformations and has been associated with good
intermediate term results. Both of the thera peutic approaches
devised for treatment of hypoplastic left heart syndrome have been
effective and the outcomes are continuing to improve with each."
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