Hypoplastic left heart syndrome is the commonest cause of death from congenital heart disease in the first week of life. There is disturbed intrauterine development of the whole left heart, possibly due to premature closure of the foramen ovale. The condition is characterised by a small left atrium, mitral valve, left ventricle and aortic root are small.
Survival in utero is due to maintenance of the systemic circulation by the right ventricle via the ductus. After birth the ductus closes and the left heart proves too small to support the systemic circulation and systemic perfusion is lost.
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