Hypoplastic left heart syndrome: Prognosis and management options

Few congenital heart malformations have raised as many surgical, ethical, social and economic issues as the therapy for infants diagnosed with hypoplastic left heart syndrome. Before the 1980s, this complex malformation was associated with 95% mortality within the first month of life. In the past two decades, palliative surgery and cardiac transplantation have become management options, in addition to comfort care for the infant. These innovations have forced parents and physicians to make difficult decisions because the long term results of the additional treatment options are not known. This article describes the current risk factors, diagnosis, treatment and outcome of infants with hypoplastic left heart syndrome. Prenatal diagnosis provides families with time for counselling and for becoming more informed about management options. Surgical therapy provides hope for the survival of these infants, but their long term outcomes are not well defined. Comfort care in either the home or hospital remains an acceptable management option. More investigations to determine the long term outcome following palliative surgery and transplantation are needed before they become the standards of care.