Surgical treatment of double inlet ventricle ("single ventricle")

The surgical techniques described are the result of an evolution over a number of years in the performance of the septation operation and the modified Fontan-Kreutzer repair for patients with double inlet ventricles. Those with associated pulmonary stenosis are best palliated by a classical Blalock-Taussig or Goretex shunt if an operation is required during the first few years of life and later, between two and four years of age, definitive repair by the modified Fontan-Kreutzer operation is advised. Although controversial, we prefer the use of a large nonvalved right atrial-pulmonary artery connection. Ventricular septation remains the best definitive surgical option when pulmonary stenosis is absent or mild. It is contraindicated by severe pulmonary vascular disease and also by less than moderate ventricular enlargement. The need for concomitant AV valve replacement and the use of an extracardiac conduit are associated with increased hospital mortality in our experience. Infants identified during the first year of life who do not have pulmonary stenosis are a difficult subset to manage. If the VSD and subaortic area is large and unobstructed, pulmonary artery banding early in life will control pulmonary vascular resistance and from this standpoint, permit these patients to become ultimately suited to a modified Fontan-Kreutzer repair. Unfortunately, ventricular hypertrophy usually results from pulmonary artery banding and has been associated with higher hospital mortality at the time of definitive repair. When pulmonary artery banding is undertaken for this subset, debanding and definitive repair seems best advised at about two years of age. Pulmonary artery banding is well known to accelerate the development of subaortic stenosis by spontaneous progressive restriction of the VSD. This results in small ventricular cavity size and increased ventricular hypertrophy, which are incremental risk factors for increased hospital mortality by either definitive procedure. When the VSD or subaortic area is narrow and the patient is identified during the first year of life, isolated pulmonary artery banding is inappropriate. The surgical options for these patients include Ebert's two-stage management program consisting of the initial placement of a loose partial septation patch with concomitant pulmonary artery banding, and later debanding and complete septation. Alternatively, a trial of primary complete septation may be warranted, or the use of a procedure consisting of division of the main pulmonary artery with distal closure and anastomosis of the proximal portion to the side of the ascending aorta, coupled with a systemic-pulmonary artery shunt.