Management of transposition of the great arteries in southern France: impact of antenatally diagnosis

  • Dr Nicolas Hugues, Children's hospital La Timone, Marseille, France
  • Dr Pascal Amedro, Arnaud de Villeneuve Hospital, Montpellier, France
  • Dr Jean-René Lusson, Gabriel Montpied hospital, Clermont-Ferrand, France
  • Dr Philippe Acar, Children's hospital, Toulouse, France
  • Dr François Bourlon, Cardio-Thoracic center, Monaco, Monaco
  • Dr Francis Rouault, St-Joseph hospital, Marseille, France
  • Dr François Heitz, Clinique Pasteur, Toulouse, France
  • Dr Alain Fraisse, Children's hospital La Timone, Marseille, France

    • Introduction
    Few multicentric data are available regarding the management of transpositions of the great arteries (TGA). We report a multi-centric experience in southern France.
    Materials and Methods
    From June 2002 to June 2007, 154 neonates presented with TGA to 8 centres.Among them, 66(42,8%) were diagnosed prenatally(Group A) and 57,2% post-natally(Group B).Group B was only studied for mortality statistics.
    Results
    An amniocentesis was performed in 37(56%) cases with one premature delivery. Standard karyotypic analysis was normal in all the cases. 53 patients (80%) were delivered in a level III maternity and the rest in a level II. An on-site surgeon was available in 25(37,8%) cases.A balloon atrial septostomy (BAS) was performed within 24 hours postnatally in 59(94%) patients.There were 2(3%) deaths prior to surgery.The surgical repair through the arterial switch operation was performed at a median age of 11(5-40) days. There were 6 postoperative deaths (9.4%) due to myocardial ischemia in 4 patients (2 with intramural coronary arteries). Factors influencing post-operative survival were: The degree of technical difficulty in re-implanting the coronary artery(p=0.006), Post-oprative ECG anomalies(p<0.0001),Time on extra-corporeal circulation(p-0.0004), Aortal clamping time(p=0.02). Factors influencing reanimation time included:Prematurity(p=0.007),Extra-cardiac anomalies(p = 0.036),Place of delivery(p=0.023) and Post-operative ECG anomalies(p= 0.005).The incidence of TGA did not appear to be affected by seasonal variations. When comparing group A to group B, there was no statistically significant difference in pre- or global operative mortality.
    Conclusion
    TGA remains undiagnosed prenatally in the majority of the cases.Intramural coronary anatomy seems to remain the main risk factor for surgical death