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