Methods: A total of 72 patients (25 males, 47 females; mean age 44.6±65.8 months; range 0 day to 255 months) with left atrial isomerism in two congenital heart diseases centers were retrospectively analyzed between July 2002 and December 2016. Clinical, radiological, electrocardiographic, echocardiographic, and angiocardiographic findings of the patients, surgical and transcatheter procedures, and postoperative follow-up data were recorded.

Results: The mean follow-up was 108±49.5 (range 12 to 173) months. Of 72 patients, four did not require surgery, while a permanent pacemaker was implanted in two patients. Of 68 patients who needed surgery, 17 were the candidates of biventricular correction, while the remaining 51 patients were the candidates of univentricular correction. The Kawashima procedure was performed in 29 patients. The pulmonary antegrade flow was left open in seven of these patients. In four patients, hepatic veins were incorporated into the Fontan circulation at the same session. In six patients, hepatic veins were directed to the pulmonary artery due to progressive cyanosis during follow-up using extracardiac conduit. Three patients died after surgery. Mortality or progressive cyanosis was not observed in any patients in whom the hepatic veins were incorporated into the Fontan circulation at the same or in another session, or in the patients in whom the antegrade flow was left open.

Conclusion: The overall prognosis is excellent in patients with biventricular physiology. However, tissue oxygenation tends to fall in patients undergoing Kawashima procedure. Therefore, incorporation of the hepatic veins into the pulmonary circulation at the same session, or leaving the antegrade flow open in suitable cases seems to be a safe approach in patients undergoing Kawashima procedure.