Tricuspid valve regurgitation in patients with hypoplastic left heart syndrome is a cause of significant morbidity and mortality. Due to the multifactorial nature of its etiology, repair of the tricuspid valve may not always address the underlying problem and fail to improve late outcome. The role of tricuspid valve repair in this condition was examined in a series of patients with severe regurgitation following a Norwood procedure. The influence of surgical technique and right ventricular function were assessed in relation to late survival and tricuspid valve function. Tricuspid valve repair was successfully performed in the majority of patients and contributed to improved late outcomes. However, patients with an initially successful repair but significant right ventricular dysfunction tended to do poorly and the function of the valve often continued to deteriorate. Those with preserved ventricular function, even with continued tricuspid regurgitation, fared much better and achieved good results with additional attempts at repair.