Abstract 15146: Increased Pre-Norwood Tricuspid Valve Annular Size and Tethering in Hypoplastic Left Heart Syndrome is Associated with Subsequent Tricuspid Valve Regurgitation and Surgery
Introduction: Tricuspid regurgitation (TR) is a major risk factor for morbidity and mortality in hypoplastic left heart syndrome (HLHS). There is limited data on the relationship between tricuspid valve (TV) function before stage 1 surgical palliation (S1P) and medium to long-term outcomes. We hypothesized that patients who develop significant TR or require TV surgery, have detectable TV abnormalities using three-dimensional echo (3DE) prior to S1P.
Methods: Eighteen selected infants with HLHS were assessed with echocardiography prior to S1P, and followed-up for the end-points of moderate/severe TR, TV surgery, cardiac transplantation or death. Two-dimensional echo was used to assess degree of TR (pre-S1P and at follow-up), mitral valve (MV) annulus, right ventricular (RV) fractional area change (FAC), RV sphericity index (SI) and RV end-diastolic area (RVEDa). TV annulus size and leaflet prolapse and tethering volumes were calculated from customized software (MathWorks, Mass) using three-dimensional spatial coordinates (TomTec, Germany).
Results: At pre S1P the HLHS infants had similar body size, RV size, shape, and function. All had mild or less TR. At follow-up (mean 18 ±14 months), 7 patients had moderate/severe TR (group A); 11 had ≤ mild TR (group B). All patients in group A had significant morbidity with 5 requiring TV surgery (vs. 0 in group B; P<0.01) and 2 progressing to transplantation (vs. 1 in group B). Four patients from group A died, as compared to 2 from group B (P=0.09). 3DE TV assessments in group A showed increased mid-systolic leaflet area (P<0.05) with a trend to increased total leaflet area (11.3 vs 9.9 cm2; P=0.1), annular area (1.7 vs 1.4 cm2; P=0.1) and reduced leaflet coaptation area (0.21 vs 0.27; P=0.11). The 3D tenting height was increased in group A (P<0.01) with tethered volumes tending to be greater (0.09 vs 0.11 ml; P=0.1). There was negligible TV prolapse in either group with prolapse volumes approaching zero. Group A had a smaller MV diameter (P=0.05).
Conclusion: Pre S1P increased TV annular size and leaflet tethering parameters, and smaller MV annulus in HLHS are associated with late TR, TV surgery and death. TV function, soon after birth, may have a role in predicting of morbidity and mortality in HLHS.
- © 2012 by American Heart Association, Inc.