title: Rapid 2-stage Norwood I for high-risk hypoplastic left heart syndrome and variants.
abstract: OBJECTIVE
  Preoperative comorbidities (PCMs) are known risk factors for Norwood stage I (NW1). We tested the hypothesis that short-term bilateral pulmonary arterial banding (bPAB) before NW1 could improve the prognosis of these high-risk patients.
  
  
  METHODS
  From January 2006 to October 2011, 17 high-risk patients with hypoplastic left heart syndrome (defined as having ≥4 of the following PCMs: prolonged mechanical ventilation; older age; sepsis; necrotizing enterocolitis; hepatic, renal, or heart failure; coagulopathy; pulmonary edema; high inotropic requirements; anasarca; weight <2.5 kg; and cardiac arrest) were identified. In addition to conventional treatment of PCMs, they underwent bPAB before NW1. bPAB was undertaken with Silastic slings and secured with ligaclips to a luminal diameter of approximately 3.5 to 4.0 mm. The patency of the ductus arteriosus was maintained with prostaglandin. NW1 was performed using a modified, right Blalock-Taussig shunt at a median interval of 8 days after bPAB. The data from these patients were retrospectively reviewed, and the 30-day mortality and 1-year survival were compared with the hypoplastic left heart syndrome population who underwent primary NW1 with <3 PCMs in the same period.
  
  
  RESULTS
  Of the bPAB patients, 5 (29.4%) died before NW1. All had ≥5 PCMs. Twelve patients (70.6%) survived to undergo NW1. One early death occurred after NW1 (8.3%). The 1-year survival rate for high-risk patients who underwent NW1 was 66.7%. The early mortality and 1-year survival for the 130 patients with <3 PCMs was 10% and 80%, respectively.
  
  
  CONCLUSIONS
  Optimizing the balance between the pulmonary and systemic blood flow with a short period of bPAB and ductal patency can improve the perioperative conditions of high-risk patients before NW1. Those who survived bPAB and underwent NW1 had early mortality and 1-year survival comparable to the standard risk category, despite the severity of their initial condition. A rapid 2-stage NW1 strategy with bPAB and prostaglandin to maintain ductal patency can avoid the risks of suboptimal palliation and vascular injuries associated with hybrid procedures.
authors.affiliation: Department of Cardiothoracic Surgery, Great Ormond Street Hospital for Children, London, United Kingdom.
authors.forename: Marcello
authors.initials: M
authors.lastname: Gomide
authors.forename: Barbara
authors.initials: B
authors.lastname: Furci
authors.forename: Branko
authors.initials: B
authors.lastname: Mimic
authors.forename: Kate L
authors.initials: KL
authors.lastname: Brown
authors.forename: Tain-Yen
authors.initials: TY
authors.lastname: Hsia
authors.forename: Robert
authors.initials: R
authors.lastname: Yates
authors.forename: Martin
authors.initials: M
authors.lastname: Kostolny
authors.forename: Marc R
authors.initials: MR
authors.lastname: de Leval
authors.forename: Victor T
authors.initials: VT
authors.lastname: Tsang
mesh_terms: D000367:Age Factors; D000527:Alprostadil / Q000008:administration & dosage; D015897:Comorbidity; D004374:Ductus Arteriosus, Patent / Q000503:physiopathology; D005260:Female; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008026:Ligation; D008297:Male; D058327:Norwood Procedures / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D011651:Pulmonary Artery / Q000503:physiopathology / Q000601:surgery*; D011652:Pulmonary Circulation; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000527:Alprostadil
keywords: 20; HLHS; NW1; Norwood stage I; Q(p)/Q(s); bPAB; bilateral pulmonary arterial banding; hypoplastic left heart syndrome; pulmonary/systemic flow ratio