Tricuspid valve repair in children with hypoplastic left heart syndrome during staged surgical reconstruction.

BACKGROUND: The purpose of this study was to determine the incidence of moderate to severe tricuspid valve regurgitation in children with hypoplastic left heart syndrome (HLHS) undergoing staged surgical reconstruction, to assess the mechanisms responsible, and to evaluate the efficacy of surgical repair.

METHODS AND RESULTS: We retrospectively reviewed clinical and echocardiographic data of 59 consecutive patients with HLHS operated on at our institution. Patients with a moderate or severe degree of tricuspid regurgitation (> or =2+) demonstrated by color flow Doppler echocardiography before the hemi-Fontan or Fontan operation who underwent tricuspid valve repair were included. Patients with HLHS and coexisting atrioventricular septal defect were excluded from the study. Severity of insufficiency was graded on a scale from 1 to 4+ by Doppler color flow mapping. Tricuspid valve morphology, degree of tricuspid valve regurgitation, and right ventricular function were assessed before and after tricuspid valvuloplasty. Eight patients (8/59; 14%) were found to have a minimum of 2+ tricuspid valve regurgitation before hemi-Fontan or Fontan operations. In five of eight patients (62%; Group A), the valve was found to be myxomatous, thickened, and redundant. There were multiple regurgitant jets in three of five (60%) patients in this group. In three of eight patients (37%; Group B), the valve had a normal echocardiographic appearance and regurgitation was the result of a lack of complete leaflet coaptation. All eight patients underwent tricuspid valvuloplasty, and all experienced a decrease in regurgitation. In five of eight (62%) patients, there was a reduction in insufficiency of two grades of severity and in three of eight (37%) patients, there was a one-grade improvement. Tricuspid valve stenosis was not documented in any of the patients after tricuspid valvuloplasty. In seven of eight (87%) patients, right ventricular function was assessed as fair before tricuspid valvuloplasty repair and improved to good in five of seven (71%) patients after the procedure. The remaining patient had good function both at baseline and after tricuspid valvuloplasty.

CONCLUSIONS: Moderate to severe tricuspid valve regurgitation is a common finding in patients with HLHS undergoing staged surgical reconstruction and can result from either abnormal valve morphology or incomplete leaflet coaptation. Tricuspid valvuloplasty during either the hemi-Fontan or Fontan stages of reconstruction carries a high success rate and is associated with improved right ventricular function.