Relevant Physiology

Pathogenesis

1. Large VSD
2. Over-riding aorta
3. Pulmonary stenosis
4. RVH

Presentation

Signs and Symptoms

• Cyanosis
  • Degree depends on pulmonary stenosis
    • severe → higher RV pressure → more R → L → more cyanosis
• Tet spells: hypercyanotic episodes in children
  • spasm of the muscle below pulmonary valves → worsened pulmonary stenosis
  • may instinctively squad → increase afterload → reverse the R-L shunt
  • can lead to
    • syncope
    • sudden death

Investigation

Hyperoxia Test (<150/>250)

Physical Exam Findings

• Auscultation: Murmur of PS/PA, SEM, PS murmur

ECG

CXR

Management