Outcomes of tricuspid valve repair in children with hypoplastic left heart syndrome.

OBJECTIVES: Tricuspid valve regurgitation (TR) is a common finding in children with hypoplastic left heart syndrome (HLHS) undergoing staged surgical reconstruction and can result from either abnormal valve morphology or incomplete leaflet coaptation due to annular dilatation. The purpose of this study was to determine the incidence of severe TR and to evaluate the effect of surgically treated tricuspid valve (TV) disease on overall survival.

METHODS: Between December 1988 and December 2012, 86 patients with HLHS underwent a Norwood procedure (mean age/weight, 11.1 days/3.5 kg). Thirty-five percent had associated congenital cardiac, structural, and genetic anomalies. Subsequently, 65 patients underwent a bidirectional Glenn procedure or a hemi-Fontan (stage II) and 42 patients a modified Fontan procedure (stage III). The mean follow-up was 6.6 years. All patients were evaluated preoperatively and postoperatively by Doppler echocardiography to determine the degree of TR.

RESULTS: At the time of discharge following the Norwood procedure, 64% (n = 47) of the patients had none/trivial, 32% (n = 24) had mild, 3% had moderate (n = 2), and 1% (n = 1) had severe TR on their post-Norwood Doppler echocardiograms. Among 74 Norwood survivors, 11 children (15%) with severe TR underwent TV repair at a median interval after the Norwood procedure of nine months (range, 2-70). There were one surgical and one late death, giving an overall mortality of 18%. The overall survival rate between TR and non-TR repair groups was not significantly different (p = 0.18). Four patients (36%) underwent reoperation for persistent TR.

CONCLUSIONS: Severe TR as a risk factor for mortality in children undergoing palliative procedures for HLHS can be diminished by successful TV procedures. Survival at intermediate follow-up of patients with successful TV procedures that eliminate severe TR and patients without a history of severe TR are similar. Further follow-up is required to determine the impact of surgically treated TR on long-term survival and the durability of surgical techniques.