Hypoplastic left heart syndrome (HLHS) represents congenital heart disease lethal during the early natural course characterized by a small left ventricle and stenosis or atresia of both the aortic and the mitral valve. Untill recently this heart lesion was considered to be inoperable. Considerable recent improvement of results of neonatal cardiac surgery, improvement of Norwood reconstructive surgery and perioperative care have improved the prognosis of these patients. Operative results depend on the presence of risk factors. Because of this fact, patients with an ascending aorta less than 2.5 mm in diameter, presence of severe tricuspid regurgitation, dysfunction of the right ventricle, severe associated lesions, chromosomal aberrations, severe acquired lesions, weight less than 2.5 kg, patients older than 6 weeks and neonates, whose parents did not agree with the surgical treatment, are not indicated for operation. Out of 8 operated neonates with this heart disease, two (25%) died during the early postoperative period. Our early experiences with the surgical treatment of HLHS are encouraging.

        Key words: congenital heart disease, hypoplastic left heart syndrome, cardiac surgery, Norwood operation,