Hypoplastic left heart syndrome (HLHS) is a common congenital heart defect that is universally fatal without surgical intervention. Two distinctly different surgical options, heart transplantation and reconstructive surgery, have been successful in treating infants with HLHS. This article reviews the anatomy and pathophysiology of HLHS. Preoperative care of infants with HLHS is discussed. The care required by infants with HLHS after heart transplantation is the same as that required by other infants who need heart transplant and has been reported. The primary focus of this report is postoperative care after staged reconstructive surgery for infants with HLHS

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