title: Improved outcomes with the comprehensive stage 2 procedure after an initial hybrid stage 1.
abstract: 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.
authors.affiliation: Heart Center at Nationwide Children's Hospital, Columbus, Ohio; Department of Cardiothoracic Surgery, Nationwide Children's Hospital, Columbus, Ohio; Department of Surgery, Ohio State University, Columbus, Ohio. Electronic address: mark.galantowicz@nationwidechildrens.org.
authors.forename: Mark
authors.initials: M
authors.lastname: Galantowicz
authors.affiliation: Heart Center at Nationwide Children's Hospital, Columbus, Ohio; Division of Cardiology and Critical Care, Nationwide Children's Hospital, Columbus, Ohio; Department of Pediatrics, Ohio State University, Columbus, Ohio.
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Yates
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D001157:Arterial Occlusive Diseases / Q000209:etiology / Q000517:prevention & control; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D007223:Infant; D007226:Infant Mortality; D008297:Male; D009820:Ohio; D011651:Pulmonary Artery / Q000503:physiopathology / Q000601:surgery; D012189:Retrospective Studies; D012307:Risk Factors; D013927:Thrombosis / Q000209:etiology / Q000517:prevention & control; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants
keywords: hybrid approach; hypoplastic left heart syndrome