Lung cancer in a young adult Fontan: what would you do?

Primary pulmonary malignancies in single-ventricle patients are rare. Improved survival following surgical palliation has resulted in a growing Fontan population whose malignancy risk remains undefined. Standard oncologic approaches, especially for adenocarcinoma of the lung, need to be considered within the context of Fontan physiology. We present our management of primary lung adenocarcinoma in an adult with tricuspid atresia status postlateral tunnel Fontan palliation, highlighting considerations for this unique scenario.