Hypoplastic left heart syndrome in the emergency department: an update

Among currently available surgical options for the first stage of surgery for infants with hypoplastic left heart syndrome (HLHS), the hybrid procedure is relatively new and less well known among primary care and emergency physicians. This procedure involves placement of a stent within the ductus arteriosus to maintain systemic blood flow and bands around both pulmonary arteries to prevent pulmonary overcirculation. As the number of infants undergoing this procedure increases, emergency physicians will likely encounter them in their practice and should be familiar with their unique physiology and potential complications.

Review various emergency department (ED) presentations and management of a patient after the hybrid procedure.

A 4-month-old male infant with HLHS who had undergone an uncomplicated hybrid procedure in the neonatal period presented to a community ED with severe metabolic acidosis and poor perfusion. He was intubated and received mechanical ventilation with an inspired oxygen concentration of 60%. Initial capillary blood gas revealed PO2 59 torr, which, in the context of his clinical presentation, was suggestive of pulmonary overcirculation with "systemic steal." Approximately 60 min after presentation to the ED, he experienced a bradycardic arrest. He expired 40 min later.

This case highlights the potential of infants who undergo the hybrid procedure for HLHS to present to the ED with high acuity and, accordingly, the importance of adding this disease process to the vast burden of knowledge facing ED physicians.