Pulmonary venous (PV) obstruction is associated with a poor prognosis, as well as
a high risk of recurrence, following surgical treatment. It can also interfere
with the successful completion of Fontan circulation in patients with complex
congenital heart disease. A case of a patient who had right isomerism (also
known as asplenia syndrome), total anomalous pulmonary venous connection
(TAPVC), and a single right ventricle is presented. Although bilateral total
occlusion of the inferior PVs was identified postoperatively, the formation of
the anastomosis and collateral vessels into the superior and middle PVs enabled
successful completion of Fontan circulation. Anastomoses and collateral flow of
the PVs were found largely in the interlobar pleura and not in the lung
parenchyma.