Hypoplasia of the left ventricle is a congenital cardiac lesion that is almost universally fatal if left untreated. Six decades of improved diagnostic modalities, greater understanding of single ventricle physiology, and earlier surgical and palliative options have given many of these patients an opportunity of surviving well into adulthood. This review will summarize these advances and focus on the anesthetic implications of this challenging disease from diagnosis to beyond the first palliative surgery.
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