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 hyp oplastic left heart syndrome (HLHS) undergoing staged surgical reconst ruction, to assess the mechanisms responsible, and to evaluate the eff icacy of surgical repair. Methods and Results We retrospectively revie wed clinical and echocardiographic data of 59 consecutive patients wit h HLHS operated on at our institution. Patients with a moderate or sev ere degree of tricuspid regurgitation (greater than or equal to 2+) de monstrated by color flow Doppler echocardiography before the hemi-Font an or Fontan operation who underwent tricuspid valve repair were inclu ded. 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 mo rphology, degree of tricuspid valve regurgitation, and right ventricul ar function were assessed before and after tricuspid valvuloplasty. Ei ght patients (8/59; 14%) were found to have a minimum of 2+ tricuspid valve regurgitation before hemi-Fontan or Fontan operations. In five o f eight patients (62%; Group A), the valve was found to be myxomatous, thickened, and redundant. There were multiple regurgitant jets in thr ee of five (60%) patients in this group. In three of eight patients (3 7%; 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 experie nced a decrease in regurgitation. In five of eight (62%) patients, the re was a reduction in insufficiency of two grades of severity, and in three of eight (37%) patients, there was a one-grade improvement. Tric uspid valve stenosis was not documented in any of the patients after t ricuspid valvuloplasty. In seven of eight (87%) patients, right ventri cular function was assessed as fair before tricuspid valvuloplasty rep air and improved to good in five of seven (71%) patients after the pro cedure. The remaining patient had good function both at baseline and a fter tricuspid valvuloplasty. Conclusions Moderate to severe tricuspid valve regurgitation is a common finding in patients with HLHS undergo ing staged surgical reconstruction and can result from either abnormal valve morphology or incomplete leaflet coaptation. Tricuspid valvulop lasty during either the hemi-Fontan or Fontan stages of reconstruction carries a high success rate and is associated with improved right ven tricular function.