title: Impact of initial shunt type on cardiac size and function in children with single right ventricle anomalies before the Fontan procedure: the single ventricle reconstruction extension trial.
abstract: BACKGROUND
  In children with single right ventricular (RV) anomalies, changes in RV size and function may be influenced by shunt type chosen at the time of the Norwood procedure.
  
  
  OBJECTIVE
  The study sought to identify shunt-related differences in echocardiographic findings at 14 months and ≤6 months pre-Fontan in survivors of the Norwood procedure.
  
  
  METHODS
  We compared 2-dimensional and Doppler echocardiographic indices of RV size and function, neo-aortic and tricuspid valve annulus dimensions and function, and aortic size and patency at 14.1 ± 1.2 months and 33.6 ± 9.6 months in subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt (MBTS) or right ventricle to pulmonary artery shunt (RVPAS).
  
  
  RESULTS
  Acceptable echocardiograms were available at both time points in 240 subjects (114 MBTS, 126 RVPAS). At 14 months, all indices were similar between shunt groups. From the 14-month to pre-Fontan echocardiogram, the MBTS group had stable indexed RV volumes and ejection fraction, while the RVPAS group had increased RV end-systolic volume (p = 0.004) and decreased right ventricular ejection fraction (RVEF) (p = 0.004). From 14 months to pre-Fontan, the treatment groups were similar with respect to decline in indexed neo-aortic valve area, >mild neo-aortic valve regurgitation (<5% at each time), indexed tricuspid valve area, and ≥moderate tricuspid valve regurgitation (<20% at each time).
  
  
  CONCLUSIONS
  Initial Norwood shunt type influences pre-Fontan RV remodeling during the second and third years of life in survivors with single RV anomalies, with greater RVEF deterioration after RVPAS. Encouragingly, other indices of RV function remain stable before Fontan regardless of shunt type. (Comparison of Two Types of Shunts in Infants with Single Ventricle Defect Undergoing Staged Reconstruction-Pediatric Heart Network; NCT00115934).
authors.affiliation: Medical College of Wisconsin, Milwaukee, Wisconsin. Electronic address: pfrommelt@chw.org.
authors.forename: Peter C
authors.initials: PC
authors.lastname: Frommelt
authors.affiliation: New England Research Institute, Watertown, Massachusetts.
authors.forename: Eric
authors.initials: E
authors.lastname: Gerstenberger
authors.affiliation: Cincinnati Children's Hospital, Cincinnati, Ohio.
authors.forename: James F
authors.initials: JF
authors.lastname: Cnota
authors.affiliation: The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Meryl S
authors.initials: MS
authors.lastname: Cohen
authors.affiliation: Medical College of Wisconsin, Milwaukee, Wisconsin.
authors.forename: Jessica
authors.initials: J
authors.lastname: Gorentz
authors.affiliation: Duke University, Durham, North Carolina.
authors.forename: Kevin D
authors.initials: KD
authors.lastname: Hill
authors.affiliation: Congenital Heart Institute of Florida/Pediatrix, Tampa, Florida.
authors.forename: J Blaine
authors.initials: JB
authors.lastname: John
authors.affiliation: Boston Children's Hospital, Boston, Massachusetts.
authors.forename: Jami C
authors.initials: JC
authors.lastname: Levine
authors.affiliation: University of Michigan, Ann Arbor, Michigan.
authors.forename: Jimmy
authors.initials: J
authors.lastname: Lu
authors.affiliation: Emory University School of Medicine, Atlanta, Georgia.
authors.forename: William T
authors.initials: WT
authors.lastname: Mahle
authors.affiliation: Primary Children's Hospital, Salt Lake City, Utah.
authors.forename: Rachel T
authors.initials: RT
authors.lastname: McCandless
authors.affiliation: Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Luc
authors.initials: L
authors.lastname: Mertens
authors.affiliation: National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: Gail D
authors.initials: GD
authors.lastname: Pearson
authors.affiliation: Medical University of South Carolina, Charleston, South Carolina.
authors.forename: Carolyn
authors.initials: C
authors.lastname: Spencer
authors.affiliation: Alfred I. DuPont Hospital for Children, Wilmington, Delaware.
authors.forename: Deepika
authors.initials: D
authors.lastname: Thacker
authors.affiliation: Columbia University Medical Center, New York, New York.
authors.forename: Ismee A
authors.initials: IA
authors.lastname: Williams
authors.affiliation: Children's Hospital of Los Angeles, Los Angeles, California.
authors.forename: Pierre C
authors.initials: PC
authors.lastname: Wong
authors.affiliation: Boston Children's Hospital, Boston, Massachusetts.
authors.forename: Jane W
authors.initials: JW
authors.lastname: Newburger
mesh_terms: D001011:Aorta / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography / Q000379:methods; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D006321:Heart / Q000502:physiology; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D058327:Norwood Procedures / Q000379:methods*; D009929:Organ Size; D013599:Systole; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000503:physiopathology; D018497:Ventricular Dysfunction, Right / Q000503:physiopathology*; D016278:Ventricular Function, Right / Q000502:physiology*
keywords: Norwood; echocardiography; hypoplastic left heart syndrome