Double inlet left ventricle.

Double inlet left ventricle is a common form of univentricular atrioventricular connection. The clinical presentation is varied depending on associated lesions and the arrangement of great arteries. Management generally involves staging toward the ultimate goal of Fontan palliation. With advances in noninvasive diagnosis, surgical and postoperative care outcomes have significantly improved in the past decade. Most patients with double inlet left ventricle can go to school, play recreationally, and are gainfully employed. Some patients continue to pose difficult and frustrating medical problems, including arrhythmias, ventricular failure, atrioventricular valve insufficiency, subaortic obstruction, protein-losing enteropathy, and plastic bronchitis.