Perioperative Care for Patients with Hypoplastic Left Heart Syndrome

: Hypoplastic Left Heart Syndrome (HLHS) is one of the most challenging congenital heart defects which require surgical interventions during neonatal period. In normal risk cohort, its surgical outcome has been improved dramatically since Dr. William Norwood reported the 1st successful case more than 30 years ago. The introduction of routine bilateral pulmonary artery banding with subsequent Norwood operation during early infantile( we call it as "rapid 2 stage Norwood operation") has brought an earlier hemodynamic stabilization after Norwood operation. However, the outcome of high risk patients, who are complicated with highly restrictive inter-atrial communication or who have significant tricuspid valve regurgitation from the early stage, is unsatisfactory even in current era. The 1st hospitalization with Norwood procedure is the beginning of staged palliations toward good Fontan circulation in the future. Very careful perioperative management is necessary to achieve survival discharge without complications.