Double Outlet Left Ventricle: A Rare and Less Understood Clinical Entity

抄録

Double outlet left ventricle is defined as a conotruncal anomaly, where the great arteries arise entirely or predominantly from the left ventricle. Diagnosis was based on commitment of more than 50% of aortic and pulmonary annulus to the left ventricle. Absence of bilateral coni, though common, is not mandatory for diagnosis. Two young infants presented with severe cyanosis due to streaming of deoxygenated blood leading to a transposition physiology. Associated pulmonary stenosis in one worsened the hypoxia. A proposed management algorithm was based on multiple anatomical and physiological features of the disease as well as the patient size. Surgical treatment plan in these patients depend on adequacy of both ventricles, routability of the ventricular septal defect, association with pulmonary stenosis and relationship of the great arteries.