[Long-term course after palliative surgery in children with a single ventricle]. / Evolution à long terme après chirurgie palliative des enfants atteints de ventricule unique.

Single ventricle or univentricular heart is a rare congenital malformation (1/10 000) which has a very poor prognosis. The cases of 68 children with single ventricles who had palliative surgery between January 1964 and December 1982 were studied. The average age of the patients at surgery was 4.41 +/- 5.8 years; 36% of the children were under 1 year of age. Surgery consisted in 48 systemico-pulmonary anastomoses and 20 pulmonary artery bandings. The global mortality was 22% (16% in the anastomosis group and 35% in the banding group). Age seemed to be an important factor: 25% mortality in children under the age of 1; 2.4% in children over 1 year (p less than 0.05). The 15 year survival rate was 43 +/- 23% overall; 56 +/- 28% in children with anastomoses and 20 +/- 28% at 8 years in children with banding. Although surgery did not appear to improve survival it probably did improve survival it probably did improve the quality of life; 73% of the survivors has satisfactory functional results and the efforts of everyday life were well tolerated. However, the evolution was complicated in 10% of cases by infection and neurological complications, and atrioventricular regurgitation was observed in 7 patients, requiring valve replacement in 2 cases. Our results are globally comparable with those of similar previously reported studies. The best published results of physiological correction of single ventricle (Fontan, septation) are relatively unsatisfactory with a mortality rate of about 35%. Therefore, palliative surgery still appears justified in these cases.