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

CXR of hypoplastic left heart syndrome
CXR AP shows cardiomegaly with mildly increased pulmonary vascularity. The tip of the umbilical arterial catheter is at the level of the T5 vertebral body while the tip of the umbilical venous catheter is in the right atrium.
CXR of hypoplastic left heart syndrome / HLHS
CXR AP shows increased pulmonary vascularity and cardiomegaly.