[Anatomic correction of double-outlet right ventricle associated with hereditary spherocytosis--a case report]

A 3-year-old boy with hereditary spherocytosis (HS) underwent anatomical correction for double-outlet right ventricle. In addition to acute injury to the red blood cells during cardiopulmonary bypass, chronic mechanical trauma related to the presence of internal conduit and transannular patch must be considered in this patient with HS. We therefore performed splencetomy prior to cardiac surgery to prevent perioperative serious hemolysis. Preoperative examination showed the anatomy was considered suitable for REV procedure. The position of the aorta was anterior, VSD was classified as subpulmonic and associated with subvalvular stenosis. Under cardiopulmonary bypass, infundibular septum was resected and intraventricular tunnel was constructed. The pulmonary artery was transected at the level of the valve commissures and translocated onto the right ventricle without transection of aorta. Right ventricular outflow tract was reconstructed with a patch bearing monocusp. No serious complication due to hemolysis occurred perioperatively. Postoperative angiocardiography revealed no stenosis in both left and right ventricular outflow tract. The patient was discharged on the 57th postoperative day and is doing well.