The relationship between congenital heart disease and scoliosis is well known. Most of these patients present deformities that can be included in the group on spine deformities associated to a congenital heart disease, and, in another patients, the fact of perform a sternotomy in the first infancy to correct partially the heart defect, predisposes to develop the spine deformity. The improvement of the life expectancy in these patients can increase the probability of being operated of non-cardiac surgery. The patients with single ventricle circulation do not have right ventricle, so that, the pulmonary blood flow depends of the central venous pressure. The prone position used for scoliosis correction could worsen the venous return and the cardiac output. We present a 17 years of female patient case, diagnosed of a kippel Feil syndrome and a complex congenital heart disease (dextrocardia, dextroapex, complete atrioventricular septal defect, double outlet right ventricle, transposition of great arteries, pulmonary stenosis and double inferior vena cava system) partially corrected by Glenn surgery at 2 years. She present a toracolumbar scoliosis that causes severe pulmonary restriction and gets worse her functional class. The patient was operated in prone position by posterior approach and the correction using pedicular screws and bars was developed. Hemodynamic invasive monitoring like Transesophageal echocardiogram (TEE) and continuous arterial pressure monitoring were used; somatosensorial evoked potentials were used too. The patient was carried to the intensive care unit hemodinamically stable. As a severe postoperative complication at the second postoperative day she present superior cava vein thrombosis that was not extended to the pulmonary artery anastomosis and that not compromise the Glenn function. She was anticoagulated with unfractionated heparin being the vein successfully recanalized. 10 days after she was discharged to the hospitalization area.