Systemic artery-pulmonary vein fistula without sequestration

Fistulae between an artery arising from the aorta and a pulmonary vein associated with absence of a branch of the pulmonary artery, but without anomalies of the bronchial tree, are exceedingly rare. We reporta case in a child who presented with a continuous vascular murmur and developed heart failure due to the left-left shunt. Left lobectomy was performed and showed that the entire left lower lobe was occupied by a huge arteriovenous fistula between an artery stemming from the aorta and the pulmonary veins.