title: Tricuspid regurgitation in hypoplastic left heart syndrome: mechanistic insights from 3-dimensional echocardiography and relationship with outcomes.
abstract: BACKGROUND
  Our purpose was to test the following hypotheses: (1) patients with hypoplastic left heart syndrome who develop significant tricuspid regurgitation (TR) or require tricuspid valve (TV) surgery in the medium term have detectable TV abnormalities by 3-dimensional echocardiography (3DE) prestage 1 palliation and (2) TR is associated with reduced survival and increased TV intervention.
  
  
  RESULTS
  Infants were prospectively studied with 3DE and 2DE prestage 1 and followed up for the end points of TR, TV surgery, transplantation, or death. From prestage 1 3DE, spatial coordinates of TV annulus and leaflets were extracted; annulus size, leaflet area, prolapse volume, tethering volume, bending angle, and papillary muscle angle were measured. TR was assessed prestage 1 and at latest follow-up. Of 70 patients, 62 (88.6%) had mild or less TR and 8 (11.4%) had moderate or greater TR prestage 1. Prestage 1 tethering volume correlated to leaflet area (r=0.736; P<0.001), annulus area (r=0.651; P<0.001), right ventricular end-diastolic area (r=0.347; P=0.003), fractional area change (r=-0.387; P<0.001), and TR grade (r=0.447; P<0.001). At follow-up, 46 (65.7%) had mild or less TR (group A) and 24 (34.3%) had moderate or greater TR (group B). Prestage 1 3DE showed greater TV tethering volume and flatter annulus in group B. Survival was better in group A.
  
  
  CONCLUSIONS
  Increased TV tethering volume and flatter bending angle prestage 1 palliation is associated with TV failure at medium-term follow-up. Increased prestage 1 tethering is related to having larger TV annulus, larger leaflet area, larger right ventricular size, and reduced systolic function. TR progression results in increased TV intervention and decreased survival.
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada. skutty@unmc.edu.
authors: Shelby
authors: S
authors: Kutty
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: Timothy
authors: T
authors: Colen
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: Richard B
authors: RB
authors: Thompson
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: Edythe
authors: E
authors: Tham
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: Ling
authors: L
authors: Li
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: Chodchanok
authors: C
authors: Vijarnsorn
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: Amanda
authors: A
authors: Polak
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: Dongngan T
authors: DT
authors: Truong
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: David A
authors: DA
authors: Danford
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: Jeffrey F
authors: JF
authors: Smallhorn
authors: From Pediatric Cardiology, Children's Hospital and Medical Center and University of Nebraska Medical Center, Omaha (S.K., L.L., A.P., D.A.D.); Stollery Children's Hospital (T.C., E.T., C.V., D.T.T., J.F.S., N.S.K.) and Department of Biomedical Engineering (R.B.T.), University of Alberta, Edmonton, Alberta, Canada.
authors: Nee Scze
authors: NS
authors: Khoo
mesh_terms: D000416:Alberta / Q000453:epidemiology; D006348:Cardiac Surgical Procedures / Q000379:methods*; D002675:Child, Preschool; D019560:Echocardiography, Three-Dimensional; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000401:mortality; D007223:Infant; D008297:Male; D009329:Nebraska / Q000453:epidemiology; D011379:Prognosis; D011446:Prospective Studies; D015996:Survival Rate / Q000639:trends; D014262:Tricuspid Valve Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery
keywords: echocardiography, three-dimensional; heart ventricles; hypoplastic left heart syndrome; pediatric cardiology; tricuspid valve