Introduction
Tetralogy Of Fallot (TOF) with absent pulmonary valve (APV) and unilateral absence of pulmonary artery (UAPA) is a rare congenital cardiac anomaly. This syndrome was first reported in 1930.1 McCaghan et al reported a 14.3% incidence of absent LPA in a group of 35 patients with APV syndrome (APVS).2 Surgical correction carries a very high mortality, with sudden cardiac deaths reported in the majority of patients.3 Construction of durable valve in RVOT with total correction can prevent future dilatation of proximal pulmonary artery (PA), and at the same time preserve RV function in the long term. We present a patient with this combination, who underwent total correction with construction of PV using a PTFE membrane.