[Transposition of the great arteries: a curable congenital heart defect?]. / Transposition des gros vaisseaux. Une malformation congenitale curable?

ABSTRACT

Transposition of the great arteries (TGA) is a common congenital heart malformation, involving the inversion of both great vessels (aorta and pulmonary artery). It is not compatible with life in the absence of surgical treatment. The prognosis of this malformation has been transformed by the development of neonatal cardiac surgery. "Anatomic" repair has been introduced in the 80s; its goal is to provide near-normal cardiac anatomy. The mid-term results of anatomic repair of TGA can be summarized as follows overall mortality is 5% (most deaths occur during the first postoperative year and are related to coronary complications), 5% of patients need reoperation for various reasons (mainly, coronary obstruction and pulmonary stenosis), major sequelae are rare (1-2%), minor sequelae are frequent (15-20%) and require strict surveillance, most patients (70-75%) are leading a normal life and can be considered as "cured". Very long-term results remain to be determined. The management of TGA includes several steps: prenatal diagnosis, planned delivery close to a pediatric cardiology center, immediate treatment with Rashkind maneuver and/or PGE1 infusion, anatomic repair during the first week of life, life-long surveillance by a congenital cardiologist. This complex multidisciplinary approach emphasizes the need for specialized centers of congenital cardiology from fetal life to adulthood.