Tricuspid Regurgitation in Hypoplastic Left Heart Syndrome

Abstract

T ricuspid regurgitation (TR) is associated with morbidity and mortality in children with hypoplastic left heart syndrome (HLHS). [1][2][3] Cross-sectional studies using 2-dimensional echocardiography (2DE), 3-dimensional echocardiography (3DE), and intraoperative surgical inspection of the tricuspid valve (TV) after stage 1 palliation have suggested multiple causative factors for TR in HLHS. These include annular and ventricular dilation, leaflet prolapse and tethering, leaflet dysplasia, papillary muscle (PM) displacement, and right ventricular (RV) dyssynchrony. 4-6 Clinical Perspective on p 772Real-time 3DE has emerged as a clinically useful tool for anatomic and functional assessment of atrioventricular valves before and after surgical repair. 7,8 Several studies have shown the added value of 3DE over 2DE in the evaluation of mitral valve disease. 6,[9][10][11][12][13][14][15][16] In pediatric subjects, real-time transthoracic 3DE generates optimal images of the TV. [8][9][10] Quantitative 3DE permits evaluation of the spatial relationships between the TV annulus, leaflets, and the supporting apparatus by measurement of tethering and prolapse volumes, leaflet and annular areas, and PM angles. 4,11 We have reported the use of quantitative 3DE to assess mechanisms of TR and demonstrated that this technique is able to detect areas of TV tethering and prolapse in HLHS. 4 Previous studies have evaluated mechanisms for TR in HLHS at or beyond the time of the second-stage palliation; however, there is a paucity of literature on TV function before 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.

Speckle-Tracking Echocardiography Improves Pre-operative Risk Stratification Before the Total Cavopulmonary Connection

“…Importantly, abnormal ventricular function contributes to atrioventricular valve dysfunction. 22 By appropriately accounting for the confounding relationship between ventricular function and atrioventricular valve regurgitation in our analysis, we may have had inadequate power to detect the association between atrioventricular valve regurgitation and LOS in our analysis.…”

Image Segmentation and Modeling of the Pediatric Tricuspid Valve in Hypoplastic Left Heart Syndrome

“…The basic three-dimensional structure of the tricuspid valve in HLHS has been shown to be associated with valve competence and with patient survival [5, 7]. The ability to rapidly, automatically, and comprehensively characterize tricuspid valve morphology from readily available transthoracic 3DE is critical to further understanding of valve dysfunction and translation to clinical application.…”

“…Systole was chosen since it is the phase of the cardiac cycle during which atrioventricular valves are closed and resist the pressure of ventricular contraction. Abnormal annular shape and leaflet profile during systole has been associated with disease in adult mitral valves [12] and tricuspid valves in HLHS [5, 7]. The images were anonymized and exported in Cartesian format with an approximate resolution of 0.7 × 0.7 × 0.5 mm 3 .…”

“…Palliative surgical intervention is often performed in three stages culminating in the Fontan procedure, which puts systemic and pulmonary circulation in series with a single functioning right ventricle. At all stages, the tricuspid valve is the only functioning atrioventricular valve, and the development of tricuspid regurgitation is understandably associated with morbidity and mortality [3–5]. Accurate assessment of tricuspid valve morphology in HLHS is therefore essential for determining the mechanism of regurgitation and potential techniques for surgical repair [6, 7].…”

“…Three-dimensional echocardiography (3DE) captures the complex geometry of the valve in real-time and reduces the subjectivity in image interpretation associated with conventional 2D echocardiography [7–9]. Moreover, 3DE can be used to quantitate morphological abnormalities that may be predictive of clinical outcome in HLHS patients even before the first stage of surgical palliation is performed [5]. Despite a potential relationship between valve morphology and outcome, there is currently no commercial system for semi- or fully automated 3D modeling of tricuspid valves and, in particular, congenitally abnormal valves such as those in HLHS.…”