Paediatrics: Hypoplastic left heart syndrome

Hypoplastic left heart syndrome

This term is used to describe a group of disorders associated with under-development of the left side heart structures. The left ventricle is small and non-functional and the right ventricle maintains both pulmonary and systemic circulations. The latter is achieved by pulmonary venous blood passing through an ASD or patent foramen ovale, or via retrograde flow through a PDA.

Clinical features

Hypoplastic left heart syndrome (HLHS) presents with early onset (days) of cyanosis and heart failure leading to collapse and death within the first few days of life. Most infants will appear sick (greyish-blue colour) with poor peripheral perfusion and weak peripheral pulses. Central cyanosis and evidence of heart failure will be present.

Treatment

Medical management aimed at maintaining the patency of the ductus is necessary to support systemic blood flow. Surgery is either palliative (2–3 stages, Norwood operation or Hybrid procedure) or definitive (heart transplantation).