Management and outcome in hypoplastic left heart syndrome

Abstract 

Hypoplastic left heart syndrome is a common cause of cardiac death in the first few weeks of life. It comprises atresia or underdevelopment of the left-sided heart structures. Over the past 20 years, Norwood and his co-workers have developed a three-stage complex palliative correction of this lesion. The first and second stages are performed within the first few months of life; the third stage, or Fontan procedure, whereby the right ventricle is connected to the systemic circulation and the systemic venous return is connected directly to the lungs without an intervening ventricle, is performed in the second to fifth year of life. Results have improved, the survival rate for stages I and II now being of the order of 70% and actuarial survival at 2–5 years being around 60%. Recent modifications of the technique comprise a right ventricle to pulmonary artery shunt rather than the modified Blalock–Taussig shunt. This is likely to impact positively on the outcome, with survival rates of 80–90% for stages I and II and actuarial survival greater than 80% at 5 years. The long-term outlook for this group of patients is likely to be that of the normal Fontan population, most or all perhaps requiring a heart transplantation later in life.

Keywords:  Hypoplastic left heart syndrome, Norwood procedure, Fontan operation, Neurodevelopmental outcome, Neonatal and infant cardiac transplantation, Left Heart Matters group