title: Achieving Benchmark Results for Neonatal Palliation of Hypoplastic Left Heart Syndrome and Related Anomalies in an Emerging Program.
abstract: BACKGROUND
  Results of surgical management of hypoplastic left heart syndrome (HLHS) and related anomalies are often compared to published benchmark data which reflect the use of a variety of surgical and hybrid protocols. We report encouraging results achieved in an emerging program, despite a learning curve at all care levels. Rather than relying on a single preferred protocol, surgical management was based on matching surgical strategy to individual patient factors.
  
  
  METHODS
  From 2010 to 2014, a total of 47 consecutive patients with HLHS or related anomalies with ductal-dependent systemic circulation underwent initial surgical palliation, including 30 Norwood stage I, 8 hybrid stage I, and 9 salvage-to-Norwood procedures. True hybrid procedures entailed bilateral pulmonary artery banding and ductal stenting. In the salvage-to-Norwood strategy, ductal stenting was withheld in favor of continued prostaglandin infusion in anticipation of a deferred Norwood procedure. Cardiac comorbidities (obstructed pulmonary venous return, poor ventricular function, and atrioventricular valve regurgitation) and noncardiac comorbidities influenced the choice of treatment strategies and were analyzed as potential risk factors for extracorporeal membrane oxygenation (ECMO) support or in-hospital mortality.
  
  
  RESULTS
  Overall hospital survival was 81% (Norwood 83.3%, hybrid 88%, "salvage" 67%; P = .4942). Extracorporeal membrane oxygenation support was used for eight (17%) patients with two survivors. For cases with obstructed pulmonary venous return (n = 10, 21%), management choices favored a hybrid or salvage strategy (P = .0026). Aortic atresia (n = 22, 47%) was treated by a Norwood or salvage-to-Norwood. No cardiac, noncardiac, or genetic comorbidities were identified as independent risk factors for ECMO or discharge mortality in a multivariable analysis.
  
  
  CONCLUSIONS
  Our emerging program achieved outcomes that compare favorably to published benchmark data with respect to hospital survival. These results reflect rigorous interdisciplinary teamwork and a flexible approach to surgical palliation based on matching surgical strategy to patient factors. With major associated cardiac/noncardiac comorbidity and antegrade coronary flow, a true hybrid with ductal stenting was our preferred strategy. For high-risk situations such as aortic atresia with obstructed pulmonary venous return, the salvage hybrid-bridge-to-Norwood strategy may help achieve survival albeit with increased resource utilization.
authors.affiliation: Division of Pediatric and Congenital Heart Surgery, The Children's Heart Center, The University of Mississippi Medical Center, Jackson, MS, USA adodgekhatami@umc.edu.
authors.forename: Ali
authors.initials: A
authors.lastname: Dodge-Khatami
authors.affiliation: Division of Pediatric and Congenital Heart Surgery, The Children's Heart Center, The University of Mississippi Medical Center, Jackson, MS, USA.
authors.forename: William Z
authors.initials: WZ
authors.lastname: Chancellor
authors.affiliation: Division of Pediatric and Congenital Heart Surgery, The Children's Heart Center, The University of Mississippi Medical Center, Jackson, MS, USA.
authors.forename: Bhawna
authors.initials: B
authors.lastname: Gupta
authors.affiliation: Center of Biostatistics and Bioinformatics, The University of Mississippi Medical Center, Jackson, MS, USA.
authors.forename: Samantha R
authors.initials: SR
authors.lastname: Seals
authors.affiliation: Division of Pediatric Cardiology, The Children's Heart Center, The University of Mississippi Medical Center, Jackson, MS, USA.
authors.forename: Makram R
authors.initials: MR
authors.lastname: Ebeid
authors.affiliation: Division of Pediatric Cardiology, The Children's Heart Center, The University of Mississippi Medical Center, Jackson, MS, USA.
authors.forename: Sarosh P
authors.initials: SP
authors.lastname: Batlivala
authors.affiliation: Division of Pediatric Cardiology, The Children's Heart Center, The University of Mississippi Medical Center, Jackson, MS, USA Division of Pediatric Critical Care, The Children's Heart Center, The University of Mississippi Medical Center, Jackson, MS, USA.
authors.forename: Mary B
authors.initials: MB
authors.lastname: Taylor
authors.affiliation: Division of Pediatric and Congenital Heart Surgery, The Children's Heart Center, The University of Mississippi Medical Center, Jackson, MS, USA.
authors.forename: Jorge D
authors.initials: JD
authors.lastname: Salazar
mesh_terms: D001021:Aortic Valve / Q000601:surgery; D019985:Benchmarking; D000082882:Bicuspid Aortic Valve Disease; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery; D006349:Heart Valve Diseases / Q000401:mortality / Q000601:surgery; D017052:Hospital Mortality; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality / Q000601:surgery*; D007223:Infant; D007902:Length of Stay; D008297:Male; D058327:Norwood Procedures / Q000379:methods / Q000401:mortality; D010166:Palliative Care / Q000379:methods*; D011183:Postoperative Complications / Q000209:etiology; D011651:Pulmonary Artery / Q000002:abnormalities / Q000601:surgery; D012307:Risk Factors; D015607:Stents; D014656:Vascular Surgical Procedures
keywords: CHD; Norwood operation; hypoplastic left heart syndrome; outcomes