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In this issue of Archives of Disease in Childhood, the Guy's group present their experience with staged reconstructive surgery for hypoplastic left heart syndrome (HLHS).1 This leading article reviews the management and outcome of this condition with special reference to this important paper and stresses the outcome from the perspective of antenatal diagnosis.
Management of HLHS
The active surgical management of HLHS has been established in the UK over the past 12 years. It comprises three operations (Norwood stages I, II, and III) that establish the right ventricle as the systemic chamber. The first stage is discussed in more detail below. The second stage is when a cavopulmonary shunt is established and the third stage is the completion of the Fontan circulation.
PREOPERATIVE PERIOD
Figure 1A summarises the situation in the preoperative period. The systemic and pulmonary circulations are both supplied by the right ventricle. The balance between flow to the lungs and flow to the body via the patent arterial duct is critical: too much pulmonary blood flow will result in systemic underperfusion and too little in hypoxaemia. A satisfactory balance between adequate oxygenation and perfusion is often achieved in the early newborn period without specific intervention, other than intravenous prostaglandin to maintain ductal patency. However, if signs of a low systemic cardiac output develop (low urine output and progressive acidosis) it will be necessary to increase the pulmonary vascular resistance by active respiratory management: it may seem counterintuitive to reduce inspired oxygen in a sick, shocked neonate but this is frequently necessary to achieve stability. Furthermore, the oxygen levels in inspired air may prove …