Abstract
Tetralogy Of Fallot (TOF) with absent pulmonary valve (APV) and
unilateral absence of pulmonary artery (UAPA) is an extremely rare
congenital cardiac anomaly. “Complete” unilateral absence implies
absence of both intrapericardial and hilar segments. Co- existence of
both free pulmonary regurgitation (PR) and decrease in the cross
sectional area for right ventricular outflow tract (RVOT) in this
substrate, are detrimental for right ventricular (RV) function. Early
intervention to preserve RV function is imperative. Durable and
competent valve in RVOT can be extremely helpful in improving the
overall prognosis. We present a case of a two year old child, TOF with
APV, absent left pulmonary artery (ALPA) who underwent RVOT
reconstruction, with a pulmonary valve (PV) made from
polytetrafluorethylene (PTFE) membrane. To our knowledge construction of
PV using Graham Nunn technique, has not been reported with this
morphology.