Tricuspid regurgitation in hypoplastic left heart syndrome mechanistic insights from 3-dimensional echocardiography and relationship with outcomes

Shelby Kutty, Timothy Colen, Richard B. Thompson, Edythe Tham, Ling Li, Chodchanok Vijarnsorn, Amanda Polak, Dongngan T. Truong, David Alan Danford, Jeffrey F. Smallhorn, Nee Scze Khoo

Research output: Contribution to journalArticle

17 Scopus citations


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. Methods and 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.

Original languageEnglish (US)
Pages (from-to)765-772
Number of pages8
JournalCirculation: Cardiovascular Imaging
Issue number5
Publication statusPublished - Sep 1 2014



  • Echocardiography
  • Heart ventricles
  • Hypoplastic left heart syndrome
  • Pediatric cardiology
  • Three-dimensional
  • Tricuspid valve

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

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