Improved outcomes with the comprehensive stage 2 procedure after an initial hybrid stage 1.

OBJECTIVE

To report our improving institutional experience with the hybrid alternative surgical strategy for the management of hypoplastic left heart syndrome, in which hybrid stage 1 is followed by a comprehensive stage 2 procedure (removal of patent ductus arteriosus stent and pulmonary artery [PA] bands, aorta and PA reconstruction, Damus-Kaye-Stansel, atrial septectomy, Glenn).

METHODS

In this Institutional Review Board-approved retrospective review of all patients undergoing a comprehensive stage 2 procedure between January 2002 and December 2014, data were compared between the pre-protocol group (n = 64; January 2002 to March 2010) and the post-protocol group (n = 55; March 2010 to December 2014). These 2 groups flank the implementation of a perioperative management protocol to prevent PA thrombosis.

RESULTS

Pre-protocol mortality was 19% (12 of 64), with the most common mode of death involving PA thrombosis in at least 7 patients, with an urgent indication for surgery and age as contributing factors. Care modifications instituted in March 2010 included avoidance of procedures on an emergent basis or in patients aged <3 months, use of a systemic PA shunt in cases of too-small superior vena cava and/or PA, completion angiogram with a low threshold for intraoperative stenting, and postoperative anticoagulation therapy for 6 weeks. There was a significant decrease in mortality (2 of 55; 4%; P = .01), PA thrombosis (0 of 55; 0%; P = .01), and use of extracorporeal membrane oxygenation (0/55 [0%] compared with 7 of 64 [11%]; P = .01) after protocol implementation.

CONCLUSIONS

Despite the technical challenges of the comprehensive stage 2 procedure, excellent outcomes are attainable. Experience coupled with an internal quality review drove the implementation of a successful perioperative management protocol.