Tetralogy of Fallot with absent pulmonary valve in an adult

Absent pulmonary valve syndrome (APVS) is seen in around 3 to 6% of tetralogy of Fallot (TOF) patients. They present in the neonatal or infantile period with respiratory compromise, cardiac failure, or cyanosis. They rarely survive into adulthood with only few reports published in literature so far. In this report, we present an adult patient with TOF and APVS, who underwent a successful repair with prosthetic valve implantation.