8th March 2024

Clinical presentation

Most adult patients with underlying TOF will have had surgical intervention with or without a prior palliative procedure. Without surgical repair, only 10% of patients survive beyond 20 years of age. Patients with previous palliative repair can present with variable clinical findings, depending on the procedure performed. Those who have undergone total repair are often asymptomatic or may present with late symptoms, which include palpitations, reduced exercise tolerance, shortness of breath, syncope or heart failure.  Post repair clinical findings may reveal: 

• Raised JVP 
• Left parasternal heave – RV hypertrophy 
• Soft/absent P2 heart sound
• Soft diastolic murmur suggestive of pulmonary regurgitation
• Pansystolic murmur in residual VSD (due to a leak in the VSD patch)
• Early diastolic murmur suggestive of aortic regurgitation 
• Continuous murmurs from aortopulmonary collateral supply