[Double discordances with ventricular septal defect and pulmonary obstruction. A study of 72 cases]

Seventy-two patients with corrected transposition of the great arteries with ventricular septal defect and pulmonary obstruction were studied. Four deaths occurred in the neonatal period and two were lost to follow-up. The remaining 66 were divided into three groups: 1) Eight patients were not operated on because the lesions were well compensated; they are all alive and doing well eight years later. 2) Thirty-eight patients were treated with palliative surgery: one or more systemico-pulmonary shunts (33 cases), total cavo-pulmonary connection (1 case) and partial cavo-pulmonary connection complementary to a shunt (4 cases). There were 3 deaths and 17 patients were lost to follow-up. However, the 18 survivors are all well seven years later. 3) The other 20 patients underwent surgery for severe hypoxia, after previous shunt in 18 cases. A conventional surgical protocol was respected in 17 cases (closure of ventricular septal defect with pulmonary disobstruction by a direct pulmonary plasty or by ventriculo-pulmonary conduit). There were 4 deaths, 2 tricuspid valve replacements, 5 complete atrioventricular blocks requiring permanent pacing, 5 lost to follow-up and 8 good results after 4 years follow-up. In the last 3 cases, "anatomical" correction was attempted by tunnelling of the left ventricle to the aorta, conduit from the right ventricule to the pulmonary artery and intra-atrial Mustard procedure: these 3 children are doing well after 1 year though one of them required permanent pacing. Therefore, there is no place for elective surgery in this malformation: when necessary, the best option is to remain palliative as long as possible: when correction is required, an anatomical correction is the best procedure.