Damus-Kaye-Stansel with cavopulmonary connection for single ventricle and subaortic obstruction

Infants with single ventricle and transposition of the great arteries with or without aortic arch obstruction have a poor prognosis due in large part to the development of systemic outflow obstruction, a frequent consequence of pulmonary artery banding. Thus, the initial palliation and long-term treatment options are critical in terms of surgical choices and timing. We report our experience with 9 patients managed by neonatal pulmonary artery banding and early debanding, a Damus-Kaye-Stansel procedure, and either a modified Glenn shunt or a modified Fontan procedure. Some evidence of subaortic stenosis developed in every patient as manifested by a resting gradient across the systemic outflow tract (21.4 +/- 4.2 mm Hg), a small ventricular septal defect relative to the body surface area (1.57 +/- 0.39 cm2/m2), and a small ventricular septal defect relative to the aortic root cross-sectional area (0.70 +/- 0.04 cm2/m2). There were 1 early death and 1 late death after the Damus-Kaye-Stansel procedure. With the exception of 1 patient, the in-hospital course of the survivors was relatively uncomplicated. Two patients with levotransposition of the great arteries have required pacemakers. None of the survivors have residual systemic outflow obstruction. There is trivial or mild pulmonic insufficiency in 5 patients, which is not progressing. One patient had mild to moderate pulmonic insufficiency but died late presumably of an arrhythmia. We conclude that neonatal pulmonary artery banding coupled with planned early debanding, a Damus-Kaye-Stansel procedure, and cavopulmonary anastomosis is a relatively low-risk course for patients with this complex physiology.