Rapid 2-stage Norwood I for high-risk hypoplastic left heart syndrome and variants

J Thorac Cardiovasc Surg. 2013 Nov;146(5):1146-51; discussion 1151-2. doi: 10.1016/j.jtcvs.2013.01.051.


Objectives: 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.

Keywords: 20; HLHS; NW1; Norwood stage I; Q(p)/Q(s); bPAB; bilateral pulmonary arterial banding; hypoplastic left heart syndrome; pulmonary/systemic flow ratio.

MeSH terms

  • Age Factors
  • Alprostadil / administration & dosage
  • Comorbidity
  • Ductus Arteriosus, Patent / physiopathology
  • Female
  • Humans
  • Hypoplastic Left Heart Syndrome / diagnosis
  • Hypoplastic Left Heart Syndrome / mortality
  • Hypoplastic Left Heart Syndrome / physiopathology
  • Hypoplastic Left Heart Syndrome / surgery*
  • Infant
  • Infant, Newborn
  • Kaplan-Meier Estimate
  • Ligation
  • Male
  • Norwood Procedures / adverse effects
  • Norwood Procedures / methods*
  • Norwood Procedures / mortality
  • Pulmonary Artery / physiopathology
  • Pulmonary Artery / surgery*
  • Pulmonary Circulation
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • Treatment Outcome


  • Alprostadil