Long-term results after physiologic repair for congenitally corrected transposition of the great arteries

We aimed to evaluate the long-term results of physiologic repair for associated lesions of congenitally corrected transposition of great arteries (ccTGA) and to provide a basis for comparison with anatomic repair for this entity.

Sixteen ccTGA patients who underwent physiologic repair from 1970 to 2000 comprise this retrospective study. Conventional Rastelli procedure was performed in 12 patients with pulmonary stenosis or atresia (PS/PA). Ventricular septal defect closure was carried out in 2 patients, atrial septal closure in 1, and tricuspid valvuloplasty in 1 without PS/PA. Mean follow-up period was 19.4 years. Long-term survival rates were assessed with respect to the presence or the absence of preoperative PS/PA and specifically in relation with the magnitude of pre- and postoperative tricuspid regurgitation (TR).

There has been no long-term mortality in the ccTGA patients without PS/PA. Twenty-year survival rate after conventional Rastelli was 71 %. Overall 20-year freedom from more than mild TR or tricuspid valve replacement was 44 %. The development of postoperative more than mild TR was significantly linked with pre-repair right ventricular enlargement (p = 0.019), but not with the magnitude of pre-repair TR (p = 0.85).

Long-term outcomes of physiologic repair for ccTGA were equivalent to those of reported anatomic repair performed in several centers during the same era. Notably, significant TR was observed in more than half of physiologically repaired patients over the 20 years after repair. The degree of pre-repair TR cannot predict the long-term function of tricuspid valve after physiologic repair.