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Hypoplastic left heart syndrome is a life threatening congenital cardiac anomaly. After a child has been diagnosed with hypoplastic left heart syndrome, parents must make life or death decisions within days of birth. Healthcare providers must provide appropriate education so that parents are able to make informed, timely decisions. Information regarding the diagnosis, treatment options, and parental decision making process for initial decision making for hypoplastic left heart syndrome are provided to guide nurses who work with these families. The challenging decision making process that parents must go through after diagnosis of hypoplastic left heart syndrome will be described.