Tricuspid regurgitation or Ebsteinoid dysplasia of the tricuspid valve in congenitally corrected transposition: is valvuloplasty necessary at anatomic repair?

Objectives: Patients with congenitally corrected transposition of the great arteries can present with tricuspid regurgitation (TR) and Ebsteinoid dysplasia of the tricuspid valve. To determine the fate of the tricuspid valve after anatomic repair and the effectiveness of tricuspid valvuloplasty, we reviewed our experience with anatomic repair of congenitally corrected transposition of the great arteries.

Methods: From 1992 to 2012, 106 patients with congenitally corrected transposition of the great arteries underwent anatomic repair. Of the 106 patients, 24 (22.6%) had moderate or greater TR before anatomic repair, 14 of whom had Ebsteinoid dysplasia. Nine patients (8.5%) had Ebsteinoid dysplasia without significant TR, and 73 patients (68.9%) had neither TR nor dysplasia.

Results: Of the 106 patients, 6 (5.6%) with TR underwent tricuspid valvuloplasty at anatomic repair, 5 with (21%) and 1 without (11%) Ebsteinoid dysplasia (P < .001). During a median follow-up period of 32 months, of the 24 patients with TR before anatomic repair, all 6 who had undergone tricuspid valvuloplasty had mild TR or less at the latest follow-up visit; 15 of the 18 (83%) without valvuloplasty had mild TR or less (P = .4) and 3 (16.7%) had moderate or greater TR. Of the 14 patients with Ebsteinoid dysplasia and TR, 5 underwent valvuloplasty and had no significant TR during follow-up; 2 of the 9 (22.2%) without valvuloplasty had moderate or greater TR (P = .51). Valvuloplasty was associated with an absolute risk of TR reduction of 16.7%, which was further reduced by 22.2% in patients with associated Ebsteinoid dysplasia.

Conclusions: Tricuspid valve function significantly improved after anatomic repair, independent of direct surgical intervention. For significant TR associated with Ebsteinoid dysplasia, valvuloplasty should be considered.