Hypoplastic Left Heart Syndrome

• Etiology: Left sided obstructive lesion
  — Spectrum of hypoplastic left atrium, mitral valve, left ventricle, aortic valve, proximal aorta leading to left sided outflow tract obstruction so systemic perfusion of aorta is through patent ductus arteriosus
• Imaging: Admixture lesion leads to cyanosis and increased pulmonary blood flow
• Imaging CXR: Cardiomegaly with pulmonary venous congestion pattern of interstial prominence and Kerley lines and pulmonary edema
• DDX:
• Complications:
• Treatment: Corrected by 3 stage Norwood repair
• Clinical:
  — Left sided obstructive lesion so neonatal ductal dependent
  — One of few causes of cardiac failure in first 24 hours of life

Radiology Cases of Hypoplastic Left Heart Syndrome