Hypoplastic left heart syndrome

Survival of infants born with hypoplastic left heart syndrome (HLHS) has steadily improved since Norwood and colleagues first reported a multistage reconstructive approach in 1983. Although several experienced centers have achieved operative survival for the Norwood procedure between 63-94%, this procedure still remains a challenging step with high mortality for many institutions. Despite successful reconstructive surgery, most deaths occur in the first 24 to 48 hours after surgery due to hemodynamic instability secondary to unpredictable rapid fall in pulmonary resistance. Therefore, many efforts to achieve a balanced circulation have focused on limiting pulmonary blood flow and increasing systemic oxygen delivery. These measures have included reduction in the size of the shunt, use of systemic vasodilators, induction of hypoxia and hypercarbia by ventilator manipulations and measurement of lactate and mixed venous satulation. Recently RV-PA shunt using non-valved polytetrafluoroethylene (PTFE) graft was applied as a first stage palliation of HLHS to prevent hemodynamic instability associated with a classic Norwood procedure. In Okayama University Hospital, there has been no hospital and late deaths since 2000.