Hypoplastic left heart syndrome is not infrequently seen in Taiwan, but all died previously. An 8 day-old-male baby suffering from hypoplastic left heart syndrome was transferred to our hospital after cardiopulmonary resuscitation. Oral prostaglandin E2 was used to maintain ductal patency and carbon dioxide was used through a respirator to control his pulmonary blood flow. He underwent Norwood operation at age 15 days under deep hypothermia and circulatory arrest. The postoperative course was smooth and occasional tachypnea was controlled by carbon dioxide inhalation through a mask. A follow up echocardiogram showed patent shunt and neoaortic pathway, good interatrial communication and mild tricuspid regurgitation. The baby survived and has fared well 2 years postoperatively.
Conclusion: We recommend carbon dioxide manipulation before and after the Norwood operation for hypoplastic left heart syndrome with increased pulmonary blood flow uncontrollable by the respirator.