Repair of double-outlet right ventricle. Experience of 13 cases

Surgery for double-outlet right ventricle (DORV) was performed in 13 patients between November 1974 and January 1979. Subaortic ventricular septal defect (VSD) was present in 11 patients, complicated forms of DORV in 2 patients and 5 patients had important concomitant cardiac defects. Six infants (mean age 0.6 years) without pulmonary stenosis (PS) required operation because of pulmonary hypertension, whereas the 7 patients with PS underwent surgery at a considerably later stage (mean age 6 years). Interventricular tunnel-repair established continuity between the systemic ventricle and great artery in 12 patients. One case with subpulmonic VSD was managed by transposition of both venous return and arterial outflow, while the use of valved external conduits was generally avoided. Atrial incision was sufficient to permit complete intraventricular repair in 8 patients, including one pulmonary valvulotomy. Important co-existing PS was otherwise treated as in cases of tetralogy of Fallot and required transannular patch grafting in 2 instances. Hospital mortality was 3/13 patients (23%) and mainly confined to serious associated cardiac malformations which were not amenable to correction. All 10 survivors are functionally improved 1.5-5 years after surgery. Clinical and invasive re-evaluation (3 patients) could not identify the development of systemic ventricular outflow tract obstruction. One patient, who underwent enlargement of a restrictive VSD, presented angiographic evidence of a moderate aortic incompetence. No other important complications were associated with the tunnel-repair and none of the 10 survivors had complete heart block.