Outcomes and risk factors associated with tricuspid valve repair in children with hypoplastic left heart syndrome.

Tricuspid valve (TV) competence is important for successful palliation of hypoplastic left heart syndrome (HLHS). We report our experience with TV repair in HLHS patients with a focus on TV and right ventricular (RV) function and associated clinical outcomes.

METHODS: From 2002 to 2012, 219 neonates with HLHS underwent the Norwood operation. Thirty patients who underwent TV repair at various stages comprised our current series cohort. Echocardiographic and clinical data were reviewed to determine the effectiveness of TV repair and outcomes of the patients.

RESULTS: Thirty patients received TV repair during Norwood (n = 4), Glenn (n = 17) and Fontan (n = 9) operations. Median age at TV repair was 188 days (range 3-1498). Preoperatively, all patients had ≥moderate TV regurgitation and 4 (13%) patients had ≥moderate RV dysfunction. After repair, TV regurgitation was none or trivial (n = 12, 40%), mild (n = 8, 27%), ≥moderate (n = 10, 33%), whereas 10 (33%) had ≥moderate regurgitation at last follow-up; ≥moderate RV dysfunction was present in 5 (17%) patients following TV repair and 10 (33%) patients at last follow-up. Competing risk analysis showed that 10 years following TV repair, 21% of patients had TV reoperation, 18% died or underwent transplantation and 61% were alive without subsequent reoperation. Overall, 10-year survival, transplant-free survival and freedom from second TV reoperation were 89%, 71% and 78%, respectively; ≥moderate RV dysfunction following TV repair was associated with diminished transplant-free survival (P = 0.0277).

CONCLUSIONS: Although TV repair is successful in reducing regurgitation in the majority of HLHS patients, outcomes are restricted by limited repair durability with recurrent significant regurgitation in one-third of the patients. RV dysfunction in these patients is progressive and a major determinant of transplant-free survival.