title: Surgical palliation strategy does not affect interstage ventricular dysfunction or atrioventricular valve regurgitation in children with hypoplastic left heart syndrome and variants.
abstract: BACKGROUND
  All 3 palliation strategies, Norwood, Sano, and Hybrid, currently used for hypoplastic left heart syndrome pose a risk of myocardial injury at different times and through different mechanisms. We sought to compare these strategies to understand longitudinal differences in interstage ventricular dysfunction and their subsequent impact on transplant-free survival and atrioventricular valve regurgitation (AVVR) as well as the relationship between adverse events and ventricular function.
  
  
  RESULTS
  Serial echocardiographic reports and clinical data were reviewed for 138 children with hypoplastic left heart syndrome who underwent stage I surgical palliation (Sano: 11; Norwood: 73; Hybrid: 54) between 2004 and 2011. Stage II palliation was achieved in 92 (67%) patients (Sano: 7; Norwood: 51; Hybrid: 34). Interstage transplant-free survival, ventricular dysfunction, and AVVR were equivalent among palliation strategies. Patients with preserved ventricular function had a higher rate of transplant-free survival and freedom from AVVR, regardless of palliation strategy. Patients who had cardiac arrest, cardiopulmonary resuscitation, or extracorporeal membrane oxygenation (adverse events) experienced more transient and persistent ventricular dysfunction compared to those without adverse events. Surgical palliation strategies were not identified as risk factors for ventricular dysfunction or AVVR.
  
  
  CONCLUSIONS
  Surgical palliation strategy does not affect mortality, interstage ventricular function, or interstage AVVR in children with hypoplastic left heart syndrome. Therefore, the different timing and mechanisms of myocardial injury among palliation strategies do not affect outcomes. Ventricular dysfunction adversely affects transplant-free survival and atrioventricular valve function. Adverse events are associated with the development of ventricular dysfunction. To improve outcomes, interstage treatment should focus on the preservation of ventricular function.
authors.affiliation: Divisions of Cardiovascular Surgery (D.C., Y.K., F.J., J.A.P., G.S.V.A., C.A.C., O.H.) and Cardiology (L.N.B., K.J.L., R.R.C., M.K.F.), The Labatt Family Heart Centre, The Hospital for Sick Children, and the Departments of Surgery and Paediatrics, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Devin
authors.initials: D
authors.lastname: Chetan
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Kotani
authors.forename: Frederic
authors.initials: F
authors.lastname: Jacques
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Poynter
authors.forename: Lee N
authors.initials: LN
authors.lastname: Benson
authors.forename: Kyong-Jin
authors.initials: KJ
authors.lastname: Lee
authors.forename: Rajiv R
authors.initials: RR
authors.lastname: Chaturvedi
authors.forename: Mark K
authors.initials: MK
authors.lastname: Friedberg
authors.forename: Glen S
authors.initials: GS
authors.lastname: Van Arsdell
authors.forename: Christopher A
authors.initials: CA
authors.lastname: Caldarone
authors.forename: Osami
authors.initials: O
authors.lastname: Honjo
mesh_terms: D001283:Atrioventricular Node / Q000473:pathology / Q000601:surgery*; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D058327:Norwood Procedures / Q000379:methods* / Q000401:mortality; D010166:Palliative Care / Q000379:methods*; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D018754:Ventricular Dysfunction / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*
keywords: Hybrid; Norwood; Sano; hypoplastic left heart syndrome; single ventricle; ventricular function