Surgical correction of hypoplastic left heart syndrome: a new approach

Hypoplastic left heart syndrome (HLHS) always results in death if not surgically treated. Approximately half of the children diagnosed with HLHS are not referred for surgery and die within days or weeks (1-3). The surgical treatment of HLHS includes three highly complex, costly surgeries that have a high mortality rate (4). The mortality rate during the first stage of the surgery ranges from 25 to 80%. The Norwood procedure, the first stage of surgical correction, uses two conventional techniques to construct a systemic pulmonary shunt. The Norwood procedure with a BlalockTaussig shunt provides systemic pulmonary blood flow but it physiologically mimics systemic aortic valve insufficiency during diastole. The Sano modification of the Norwood procedure consists of placing a tube between the systemic ventricle and the pulmonary arteries. This technique prevents diastolic systemic pulmonary blood reflow but causes ventricular reflux, leading to volume overload. This overload (associated with the ventriculotomy) may lead to ventricle dysfunction and arrhythmia. Both techniques necessarily use an artificial tube, which increases the risk for thrombosis or stenosis (5-9). Moreover, creating the neoaorta is a laborious procedure that involves the prolonged use of extracorporeal circulation (ECC). A hybrid approach simplifies the surgery by maintaining the patent ductus arteriosus (DA) with a stent implantation (10,11) or through the prolonged use of prostaglandin E1 (12), associated with the banding of the pulmonary branches. However, the results of this approach have proven unsatisfactory (13,14).