[Current Surgical Treatment for Patients with Hypoplastic Left Heart Syndrome, from the Viewpoint of Emergent Operation].

Hypoplastic left heart syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. Since the 1st successful surgical repair was reported by Norwood et al. more than 30 years ago, some modifications in surgical maneuver [e. g. introduction of right ventricule (RV)-pulmonary artery (PA) shunt] and improvement of perioperative management have brought better outcome for this patient cohort. Recent years our institute have changed the treatment strategy from primary Norwood operation during neonatal period to bilateral PA banding and subsequent Norwood operation during early infantile (we call it as "rapid 2 stage Norwood operation"). Indeed, the introduction of this new strategy has lead to earlier hemodynamic stabilization after Norwood operation. Although these operations are performed electively for most of HLHS patients, emergent operations are necessary if they have restrictive inter-atrial communication with sever pulmonary congestion, or if their patent ductus arteriosus (PDA) s tend to close in spite of prostagrandine infusion, or if pulmonary over circulation develops so rapidly. Recently more than half of HLHS patients are diagonosed in fetal. The advances in fetal diagnosis allow us to find the patients earlier and to assess the severity of the disease. Most of HLHS patients who required emergent operation just after their birth are diagnosed in fetal. However, their surgical outcome is unsatisfactory so far. Treatment for these HLHS patients has become the next challenge.