| Abstract: | Patients who underwent only Glenn procedure after being deemed unsuitable candidates for Fontan completion are not small in number, and may develop arterio‐venous (AV) pulmonary malformations during the follow‐up period. We present the case of a 17‐year‐old woman with severe systemic desaturation 13 years after Glenn procedure and repair of total anomalous pulmonary venous return. Among other anomalies, the patient disclosed hemiazygos continuation to a persistent left superior vena cava. This case supports the concept that returning flow from the abdominal vein plays an important role in the well‐balanced growth of the pulmonary artery and in the inhibition of the pulmonary AV malformation. |
