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.