Outcomes in children with hypoplastic left heart syndrome undergoing open fundoplication.

BACKGROUND/PURPOSE: Some institutions recommend early fundoplication in patients with hypoplastic left heart syndrome (HLHS) with signs of gastroesophageal reflux disease because of the risk of reflux-related cardiac events. However, their cardiac physiology may impose high perioperative morbidity and mortality. Therefore, we reviewed our experience with fundoplication in this population to allow for assessment of the risk-benefit ratio.

METHODS

A retrospective review of patients with a diagnosis of HLHS who underwent a fundoplication from January 1990 to July 7, 2009, was performed. All patients underwent open fundoplication between first and second stages of cardiac repair.

RESULTS

Thirty-nine patients were identified. There were 3 intraoperative complications: hemodynamic instability (n = 2) and a pulmonary hypertensive crisis requiring extracorporeal membrane oxygenation and termination of the procedure (n = 1). There were 27 postoperative complications in 16 patients. There were 2 deaths (4%) within 30 days, and there were 9 deaths (23%) in patients between their first and second stage of cardiac repair during the study period.

CONCLUSIONS

Noncardiac surgical procedures in patients palliated for HLHS have a high morbidity and mortality. We recommend that routine fundoplication in this population should only be performed under prospective protocols until the relative risk of operation vs risk of reflux is delineated.