A neonate in respiratory distress has echocardiographic evidence of hypoplastic left heart syndrome (HLHS). The following is/are true:

a. Initial management should include prostaglandin infusion

b. Ventilatory adjustment should maintain PaCO2 at approximately 40 mmHg

c. Survival depends on sustained patency of the ductus arteriosus

d. Cardiac transplantation for HLHS requires inclusion of the donor aortic arch

e. Reconstruction for HLHS converts the pulmonary artery into the main outlet for a functional single ventricle (Norwood)

The neonate with HLHS has a severely underdeveloped left ventricular and aortic arch and is dependent on patency of the ductus which is facilitated by prostaglandin infusion. Ventilator adjustment to reduce supplemental oxygen and maintain PCO2 of 40 mmHg avoids excessive pulmonary flow. The options for treatment include cardiac transplantation which requires a donor aortic arch and reconstruction by the Norwood procedure which converts the pulmonary artery into the main outlet for a functional single ventricle.