page_title: Hypoplastic left heart syndrome
section_title: Signs and symptoms
hierarchical_section_title: Hypoplastic left heart syndrome / Signs and symptoms
caption_attribution_description: English: Hypoplastic left heart syndrome Српски / srpski: Sindrom hipoplazije levog srca
context_page_description: Hypoplastic left heart syndrome is a rare congenital heart defect in which the left side of the heart is severely underdeveloped. It may affect the left ventricle, aorta, aortic valve, or mitral valve.
context_section_description: Closing of the ductus arteriosus in a heart that is severely underdeveloped on the left results in cyanosis and respiratory distress which can progress to cardiogenic shock and death. The first symptoms are cyanosis that does not respond to oxygen administration or poor feeding. Peripheral pulses may be weak and extremities cool to the touch.
  HLHS often co-occurs with low birth weight and premature birth.
  In neonates with a small atrial septal defect, termed "restrictive", there is inadequate mixing of oxygenated and deoxygenated blood. These neonates quickly decompensate and develop acidosis and cyanosis.
  On EKG, right axis deviation and right ventricular hypertrophy are common, but not indicative of HLHS. Chest x-ray may show a large heart (cardiomegaly) or increased pulmonary vasculature. Neonates with HLHS do not typically have a heart murmur, but in some cases, a pulmonary flow murmur or tricuspid regurgitation murmur may be audible.
  Co-occurring tricuspid regurgitation or right ventricular dysfunction can cause hepatomegaly to develop.
caption: Infant with cyanosis due to hypoplastic left heart syndrome
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