Tricuspid Regurgitation In Hypoplastic Left Heart Syndrome: Mechanistic Insights On Tricuspid Valve Tethering And Relationship With Outcomes
Background—Our purpose was to test the following hypotheses: (1) Patients with hypoplastic left heart syndrome (HLHS) who develop significant tricuspid regurgitation (TR) or require tricuspid valve (TV) surgery in the medium-term, have detectable TV abnormalities by three-dimensional echocardiography (3DE) pre-stage 1 palliation; (2) TR is associated with reduced survival and increased TV intervention.
Methods and Results—Infants were prospectively studied with 3DE and 2DE pre-stage 1, and followed-up for the end-points of TR, TV surgery, transplantation or death. From pre-stage 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 measured. TR was assessed pre-stage 1 and at latest follow up. Of 70 patients, 62 (88.6%) had ≤mild TR; 8 (11.4%) had ≥moderate TR pre-stage 1. Pre-stage 1 tethering volume correlated to leaflet area (r=0.736; p<0.001), annulus area (r=0.651; p<0.001), right ventricular (RV) 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 TR (group A), 24 (34.3%) had ≥moderate TR (group B). Pre-stage 1 3DE showed greater TV tethering volume and flatter annulus in group B. Survival was better in group A.
Conclusion—Increased TV tethering volume and flatter bending angle pre-stage 1 palliation is associated with TV failure at medium-term follow-up. Increased pre-stage 1 tethering is related to having larger TV annulus, larger leaflet area, larger RV size and reduced systolic function. TR progression results in increased TV intervention and decreased survival.
- tricuspid valve
- three-dimensional echocardiography
- pediatric cardiology
- right ventricle
- hypoplastic left heart syndrome
- adult congenital heart disease
- Received September 6, 2013.
- Accepted July 24, 2014.