Risk factors for adverse outcome after superior cavopulmonary anastomosis for hypoplastic left heart syndrome

Outcome of staged palliation for hypoplastic left heart syndrome (HLHS) has improved over the past decades. We sought to evaluate the outcome of the second palliative procedure, the superior cavopulmonary anastomosis (SCPA), in a single-centre cohort and to identify risk factors for adverse outcome.

Full data on all 119 HLHS patients who underwent SCPA in our centre between January 1996 and December 2007 were analysed.

Early adverse outcome (death or cardiac transplant within 30 days after surgery or before hospital discharge) was 3.4%. Late adverse outcome (death or transplant after hospital discharge but before the next operative procedure) was 8.7%. Postoperative complications occurred in 30% of patients (n = 36), with transient arrhythmia (n = 11; 9%) and pulmonary artery stenosis or thrombosis (n = 10; 8%) being the most common. The presence of more than moderate tricuspid valve regurgitation after surgery proved to be a strong predictor of late adverse outcome (odds ratio (OR) 16.5 (4.4-62.6), P < 0.001). SCPA at less than 4 months of age did not increase the risk for adverse outcome (OR 1.2 (0.4-3.6), P = 0.78) but increased the risk for postoperative complications (OR 6.3 (2.3-14.9), P < 0.001).

SCPA can nowadays be performed in HLHS patients with low mortality. However, more than moderate tricuspid valve regurgitation should be targeted at surgery as it is a risk factor for adverse outcome such as death or need for cardiac transplant. SCPA should ideally be performed in children older than 4 months to minimise the risk of postoperative complications.