Hypoplastic Left Heart Syndrome

• Occurs in 1% of all CHD
• Presents as critically ill newborn with shock, acidosis and evidence of CHF
• Hypoplasia/atresia of mitral/aortic valves, left ventricle, aortic arch and ascending aorta
• Systemic cardiac output depends on patent ductus
• Symptoms are less severe if there is a large ASD and PDA

• EKG shows RVH
• CXR shows cardiomegalyand pulmonary edema
• Correction: Norwood, Glenn and Fontan
  • Norwood is used specifically for HLHS
    • Stage 1: proximal main pulm artery is joined to aorta (like a huge PDA) and the aortic arch is reconstructed; then a modified BT shunt (R subclavian to R pulm artery) to provide pulmonary blood flow
    • Stage 2: bidirectional glenn shunt (SVC is attached to the RPA)
  • Fontan: used to separate systemic and pulmonary circulations in patients with functionally single ventricles (tricuspid atresia, HLHS): Glenn shunt, then RA and/or IVC connected to pulmonary arteries via conduits
• Differential diagnosis includes sepsis, hypoglycemia, severe anemia (eg. hydrops)