Absence of left pulmonary artery in tetralogy of Fallot with absent pulmonary valve syndrome.

The rare combination of tetralogy of Fallot (TOF), absent pulmonary valve syndrome (APVS), and absent left pulmonary artery (ALPA) is reviewed herein. Children with TOF with APVS and ALPA should be closely monitored, even if they are asymptomatic or mildly symptomatic, and should undergo elective surgery at the end of infancy.