Methods: Between January 2010 and October 2014, a total of 34 patients (17 males, 17 females; median age 2.64 months; range 1 day to 24 years) with double-inlet left ventricleventriculoarterial discordance were analyzed retrospectively. The demographic characteristics of the patients, echocardiographic and hemodynamic measurements, invasive, surgical procedures performed and their outcomes were reviewed.

Results: Of the patients, 24 had pulmonary hypertension with arch obstruction in eight of them. Eight had pulmonary stenosis, and two had pulmonary atresia. None of the patients had outflow obstruction as assessed by echocardiography. Surgery was performed in 25 patients. Of these, 14 patients underwent initial pulmonary artery banding procedure and four of these also underwent arch reconstruction. Bidirectional cavopulmonary connection was performed in six patients, aortopulmonary shunt in four patients, and the Norwood-type operation in one patient. The median follow-up was 9.96 months (range 0.24 to 53.88 months). During follow-up, 12 patients underwent a bidirectional cavopulmonary connection. Systemic outflow restriction did not develop in any of the patients who were initially palliated with pulmonary artery banding with or without arch reconstruction and proceeded to the bidirectional cavopulmonary shunt stage.

Conclusion: Based on our study findings, although the primary treatment strategy in patients with double-inlet left ventricleventriculoarterial discordance displays a great variability, aortopulmonary shunting or ductal stenting can be performed in patients with restricted pulmonary blood flow, pulmonary artery banding with or without arch reconstruction in patients with unrestricted pulmonary blood flow. The Norwood type operation can be performed initially in patients with bulboventricular foramen restriction.