Absent pulmonary valve syndrome and L-transposition of the great
arteries are rare congenital anomalies. To our knowledge, patients with
both have not been previously reported. We present a case of surgical
success in the patient with absent pulmonary valve, L-transposition of
the great arteries, dextrocardia, double outlet left ventricle,
hypoplastic anatomical right ventricle, valvular aortic stenosis and
tracheomalacia. We performed anatomical left ventricle to pulmonary
artery shunt using an 8-mm expanded polytetrafluoroethylene
bicuspid-valved conduit, pulmonary artery plication, and tracheostomy.
The patient was discharged in a stable condition while waiting for the
Glenn procedure until getting her parent's permission.