Background: The aims of this study were to investigate the dynamic changes in the vena contracta (VC) and proximal isovelocity surface area (PISA) through systole in patients with hypoplastic left heart syndrome and tricuspid regurgitation and to identify the stage of systole (early, mid, or late) in which VC and PISA radius are optimal. Methods: Twenty-eight patients with hypoplastic left heart syndrome were prospectively studied using continuous two-dimensional (2D) and three-dimensional (3D) echocardiography. Two-dimensional VC width, 3D VC area, and PISA radii (2D and 3D) were measured frame by frame throughout systole. The maximal 2D VC width, 3D VC area, and PISA radii in the first, middle, and last thirds of systole were compared, and correlations were explored with 3D tricuspid annular areas, right atrial volumes, and right ventricular volumes. Results: In all, 35 data sets that met inclusion criteria were analyzed. On frame-by-frame analysis, maximal 2D VC width and 3D VC area were found in the first third of systole in 17% and 20% of studies, in the second third in 34% and 31%, and in the final third in 49% and 49%. Similarly, the maximal 2D and 3D PISA radii were found in the first third of systole in 26% and 17% of studies, in the second third in 28% and 34%, and in the final third in 46% and 49%. Conclusions: In hypoplastic left heart syndrome, detailed temporal analysis of tricuspid regurgitation- associated VC and PISA by 2D and 3D echocardiography reveals no reliable pattern predicting when in systole these parameters peak. Frame-by-frame measurement is necessary for identification of maximal VC and PISA radius on 2D and 3D color Doppler echocardiography because the severity of tricuspid regurgitation could be underestimated because of temporal variability in VC and PISA. (J Am Soc Echocardiogr 2021;34:877-86.)

BACKGROUND:Twenty-five percent of patients with hypoplastic left heart syndrome (HLHS) require tricuspid valve (TV) repair. The location of tricuspid regurgitation (TR) is important in determining the type of repair performed. Studies using three-dimensional echocardiography (3DE) have reported a high incidence of error on two-dimensional echocardiography (2DE) for the identification of TV leaflets. The aim of this study was to compare assessment of TR on 3DE and 2DE in patients with HLHS (jet location, TR grade, and reproducibility). METHODS:A retrospective, single-center review was performed. Fifty-six patients with HLHS with available two-dimensional and three-dimensional echocardiograms, and mild or greater TR, were included. TR location, grade, vena contracta area, and TV annular diameter were measured on 2DE and 3DE. Reproducibility was assessed by blinded reviewers. RESULTS:Three-dimensional echocardiography identified the primary jet location as central (57%) followed by anteroseptal (36%). There was poor agreement between findings on 3DE and 2DE for jet location (κ = 0.05; 95 CI, -0.08 to 0.19). Interobserver reproducibility for location on 3DE was excellent (κ = 0.8), whereas reproducibility for 2DE was poor (κ = 0.32). The most common jet location pre-Norwood and pre-Glenn was central (70%), whereas pre-Fontan and post-Fontan, jet location was central (45%) and anteroseptal (48%). Vena contracta area on 2DE correlated moderately with vena contracta area on 3DE (r = 0.60, P < .0001). TV annular diameters on 2DE and 3DE for lateral (r = 0.85, P < .0001) and anteroposterior (r = 0.74, P = .001) dimensions were strongly correlated. CONCLUSIONS:In children with HLHS, assessment of TR location on 2DE had poor agreement with assessment on 3DE and was poorly reproducible. In contrast, TR jet location on 3DE was highly reproducible. Pre-Glenn, a central TR jet was the most common, while post-Glenn, central and anteroseptal locations were equal, highlighting the importance of preoperative identification of TR jet location in patients with HLHS.

BackgroundChildhood cancer survivors show evidence of diffuse myocardial fibrosis that is related to exercise capacity. The mechanism of reduced exercise tolerance in anthracycline cardiotoxicity remains unclear. We explored the determinants of exercise intolerance by evaluating left ventricular (LV) distensibility and functional reserve.MethodsPatients (n = 22) and healthy controls (n = 10) underwent two‐dimensional echocardiography while supine, upright, and during cycle exercise. LV distensibility was measured as the change in end‐diastolic cavity area (EDCA) from supine to the upright position. LV functional reserve was assessed during peak exercise, and measured as the exercise‐induced change in systolic circumferential strain rate (SR) and early‐diastolic SR (EDSR). The peak rate of oxygen consumption was measured by indirect calorimetry.ResultsMedian age of patients was 16 years (range 8–19) and controls 14 years (range 8–19). Median time since anthracycline therapy was 6 years (range 2–16). Peak oxygen consumption was significantly lower in patients compared to controls (35 ml/kg/min [28–60] vs. 45 ml/kg/min [44–53], P = 0.005). Transitioning from the supine position to the upright position caused a similar reduction in LV EDCA, suggesting similar LV distensibility between patients (–22% [–46 to –4]) and controls (–20% [–46 to –3], P = 0.3). However, during exercise, both systolic SR and EDSR reserve were significantly impaired in patients (∆SR: 93% [14–308], ∆EDSR: –4.5% [–88 to 121]) compared to controls (∆SR: 128% [54–230], P = 0.046; ∆EDSR: 74% [22–234], P = 0.02).ConclusionsOur findings suggest that impaired LV contractility and functional reserve play a role in the reduced exercise capacity in anthracycline cardiotoxicity rather than LV distensibility.

Interrupted aortic arch is a rare condition with typical presentation within the first few weeks of life, as the circulation is dependent upon patency of the arterial duct. Most cases are associated with intracardiac anomalies, the most common being a ventricular septal defect with some degree of hypoplasia and/or obstruction of the left ventricular outflow tract. Presentation beyond infancy is uncommon, and suggests the presence of well-developed collateral circulation. This case of childhood presentation of interrupted aortic arch and intact ventricular septum highlights the very unusual finding of bilateral collateral arteries consistent with persistent carotid ducts. Cardiac MRI angiography with three-dimensional reconstruction defined not only the site of interruption in the aortic arch but also the entire collateral circulation.

On-pump coronary revascularization should be our preferred surgical revascularization strategy

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.

Background: Left ventricular (LV) rotation is an important component of cardiac function. There is little data on the comparison of Doppler speckle tracking with other invasive techniques for measuring LV rotation in the beating heart. We hypothesized that Doppler speckle tracking would compare favorably with data from an optical device that has previously been shown to measure LV rotation in the beating heart.

Background: Neonates with certain forms of severe congenital heart disease (CHD) diagnosed prenatally might have better outcomes in comparison with those diagnosed after birth. The proportion of prenatally detected neonates with severe CHD and the effect of prenatal diagnosis on clinical outcomes have not been previously investigated in Canada.Methods: We retrospectively studied infants in Alberta, Canada, who required surgical or catheter intervention for CHD at younger than 1 year of age, between January 2007 and December 2010, and pregnancy terminations affected by CHD.Results: Of the 374 subjects identified (327 infants, 47 pregnancies with termination), 188 (50%) were detected prenatally. Failure of prenatal diagnosis was associated with anomalies not involving the 4-chamber view on ultrasound (odds ratio, 1.86; 95% confidence interval, 1.48-2.35; P < 0.001) and region of residence (P = 0.04). Prenatal detection was associated with fewer days to hospital admission (P < 0.001), fewer days to surgery (P = 0.003), and greater use of prostaglandins (P = 0.001). Infants diagnosed prenatally who underwent surgery within 15 days of age had higher preductal O-2 saturations (P = 0.04), fewer days to admission (P = 0.03), and less frequently required preoperative intubation (P = 0.004), and inotropes (P = 0.001). Pregnancy termination occurred among 49% of fetuses detected before 24 weeks' gestation.Conclusions: Only 50% of fetuses and/or neonates with severe CHD managed in Alberta have a prenatal diagnosis. The likelihood of prenatal detection is influenced by the status of the 4-chamber view on ultrasound and the region of maternal residence indicating heterogeneous access to fetal echocardiography within Alberta. Prenatal detection might improve clinical outcomes for neonates with severe CHD.

Background: The long-term prognosis of hypoplastic left heart syndrome is limited by progressive right ventricular dysfunction. The aim of this study was to determine the trends in single right ventricular systolic function between staged palliative surgeries using speckle-tracking and conventional echocardiography.Methods: There were 76 patients with functionally single right ventricles at the (1) pre-Norwood (n = 26), (2) pre-bidirectional cavopulmonary anastomosis (BCPA; n = 19), (3) pre-Fontan (n = 16), and (4) post-Fontan (n = 15) stages, compared with 30 controls of similar ages. Speckle-tracking-derived longitudinal and circumferential strain and strain rate, postsystolic strain index, and mechanical dyssynchrony index were compared with conventional measures of ventricular function. Differences between stages were analyzed using analysis of variance (P < .05).Results: Strain rate was highest at the pre-Norwood stage and decreased at the other stages (longitudinal P < .0001, circumferential P = .0002), as opposed to controls, in whom strain rate was maintained. Longitudinal strain was significantly decreased at the pre-BCPA stage compared with the pre-Norwood stage (P = .004), but circumferential strain was maintained, resulting in a corresponding decrease in the ratio of longitudinal to circumferential strain, which failed to resemble that of controls. Longitudinal (P = .003) and circumferential (P = .002) postsystolic strain indices were greatest at the pre-BCPA stage.Conclusions: A decline in contractility occurred at the pre-BCPA stage. Although there was evidence of adaptation of the single right ventricle, this failed to resemble the normal left ventricle and may be insufficient to handle the chronic volume load or insult from previous surgery. These findings suggest an intrinsic inability of the single right ventricular myocardium to fully adapt to chronic systemic pressures.

s S97 BACKGROUND: We have previously shown that infants of diabetic mothers (IDM) have increased aortic stiffness in the first 6 months of life, and aortic stiffness is related to poor maternal glycemic control in pregnancy. Increased left ventricular (LV) posterior (LVPW) and interventricular septal (IVS) wall thickness is observed in IDM in late gestation and early infancy, predominantly in mothers with worse glycemic control. Although it is generally believed that the LV hypertrophy in IDM regresses after the first 3 months, we have recently shown that hypertrophy persists even in late infancy. In this study we sought to explore the relationship between aortic stiffness and LV hypertrophy in IDM. We hypothesized that LVPW and IVS wall thickness would be greater in infants with greater aortic stiffness. METHODS: Diabetic pregnancies were prospectively recruited in the mid trimester. Their infants were evaluated in infancy by echocardiography. LVPW and IVS wall thickness was measured by m mode and measurements were compared to normative data for the calculation of z scores based on body surface area. Aortic wall stiffness was assessed with echocardiography, using 2D and pulsed Doppler interrogation of the ascending aorta and distal arch. Aortic pulse wave velocity (PWV) was calculated as the distance from the ascending aorta to distal arch, divided by the time interval from the QRS to the onset of flow for the two interrogated sites (m/s). RESULTS: Twenty-one IDM were investigated at a median age of 7 weeks (range 2-48 weeks). Mean aortic PWV was 4.7 1.7m/s, and the mean IVS and LVPW z scores were 1.3 1.2 and LVPW 1.4 1.1, respectively. Although no relationship was observed between PWV and IVS, there was a weak but positive correlation between aortic PWV and LVPW z score (r1⁄40.50, p1⁄40.027). CONCLUSION: Our study suggests that a potential relationship exists between the degree of stiffness of the aorta and LV wall thickness. This could be due to a common prenatal exposure (hyperglycemia) or to a causal relationship (greater aortic stiffness leading to evolution or persistence of LV hypertrophy). Further studies are underway to determine the etiology of increased aortic stiffness and LV hypertrophy and the natural course of these changes. 025 PREVALENCE AND SIGNIFICANCE OF LATE GADOLINIUM ENHANCEMENT IN CHILDREN AND ADOLESCENTS WITH HYPERTROPHIC CARDIOMYOPATHY L Grosse-Wortmann, J Windram, D Andreea, S Yoo, L Mertens, D Wong, L Benson

Background: Three-dimensional echocardiography (3DE) is an accurate and informative imaging modality routinely used in pre-operative assessment for surgical planning. There is however little information on 3DE role in early post-operative valve failure assessment. This is a report on our 5 years experience of 3DE in early post-operative mitral/left atrioventricular valve (MV/LAVV) failure assessment, its impact on our understanding of its mechanism and the clinical decision for returning to the operating theatre for re-repair. Methods and results: We retrospectively reviewed all patients chart and echo with MV/LAVV surgery between 2008 and 2012, excluding primary repair of atrioventricular septal defect (AVSD) and primary valve replacement. Early valve failure (EVF) is defined as moderate or greater MV/LAVV regurgitation within 30 days post repair. There were 132 MV/LAVV surgeries consisting of repaired complete AVSD (n=85), congenital MV abnormality (n= 17) and primum AVSD (n= 16). Pre operative moderate or greater regurgitation was present in 55 (42%) and post op EVF in 33 patients (25%). 3DE were performed in 22 (67%) of 33 EVF with 8 patients returning to the operating room for re-repair due to poor clinical progress in intensive care unit. Six of 8 re-repair were infants. 3DE accurately identified the mechanism of regurgitation such as suture dehiscence at base of cleft, leaflet tears and patch failure (see Figure). Four of 8 patients MV/LAVV regurgitation improved to mild or less regurgitation at follow-up (median 2 months), 3 remained unchanged and 1 subsequently died from intractable heart failure despite MV/LAVV regurgitation improving from severe to moderate. Conclusion: Although patient returning for re-repair of EVF were mainly driven by poor clinical progress in intensive care. 3DE accurately identified the mechanism of early post-operative valve failure thus facilitating surgical planning and decision for re-repair.

Atrioventricular valve regurgitation is a major cause of morbidity and mortality in children with congenital heart disease. The impact of changes in loading conditions and inotropy on valvular regurgitation and function is poorly understood. Unlike previous animal models assessing mitral valve (MV) function, we used high spatial and temporal resolution three-dimensional echocardiography (3DE) to investigate the effect of changes to loading conditions and inotropy on normal MV function in an in-vivo animal model without instrumentation on the MV. Normal MV in 12 animal hearts (8 pigs; 4 dogs) were studied in an open chest model. All animals had normal systolic function and no MV regurgitation. Epicardial 3DE was performed using high spatial and temporal resolution (mean frame rate 55 Hz) settings, concurrent with invasive hemodynamic assessment during 4 conditions: reduced preload (external constriction of IVC), increased preload (fluid bolus), increased afterload (external constriction of aorta) and increased contractility (dobutamine) with return to baseline hemodynamics between each condition. Mitral valve function was analyzed offline using commercially available 4D-MV analysis software from Tomtec Inc. We measured MV annulus diameter, area, tenting height and volume, and leaflet area at mid-systole. The results were normalized between species and size and reported as percent change from baseline in each subject, with comparison between baseline and each condition using paired t-test. Reduction in ventricular preload decreased MV annulus size (Antero-posterior 5 % and commissural diameter 8 %), (p<0.05); MV annulus area 13 %( p<0.05). There were concurrent reduction in measured anterior leaflet area 17% (p<0.01), tenting height 20% and volume 31% (p<0.01). With inotropy, there was a reduction in tenting height 14% (p<0.01) with no change in annular size. Consistent with increased contractility, the maximum annular displacement velocity 44% (p<0.05) was increased. Effects of increased preload and afterload did not show significant difference from baseline. In the normal MV, reduction in preload had the greatest impact on MV function with probable improvement of leaflet coaptation, evidenced by decreased annular size and decreased measured atrial surface area of the anterior leaflet. Increased ventricular contractility reduced MV leaflet tenting height without significant impact on leaflet coaptation area, whilst the MV appears somewhat immune to a significant change in function by increase in preload and afterload. These findings support the concept that MV function is sensitive to reduction in preload and therapy to reduce ventricular preload is potentially beneficial in reduction of AV valve regurgitation.

The majority of unbalanced atrioventricular septal defect (UAVSD) cases undergo single ventricle palliation. Associated significant atrioventricular valve regurgitation (AVVR) results in increased morbidity and mortality. Our initial observations indicate that abnormal leaflet tethering may be one important mechanism of AVVR. Our hypothesis is that abnormal leaflet tethering is responsible for progressive AVVR in some patients with UAVSD. We retrospectively analyzed the initial and pre-Glenn echocardiograms of 40 consecutive patients with unbalanced AVSD requiring single ventricle palliation. Patients were deemed to have severe AVVR if the sum of vena contracta to dominant valve annulus ratio was ≥ 0.33. We measured dominant AVV tenting height, annular to leaflet angle, valve annulus and combined atrial area, indexing to patient size where appropriate. Univariate and multivariate analysis of variables to predict progression of AVVR was performed. Within the cohort, 21 patients had minimal AVVR (group A) and 19 severe AVVR at mean follow-up of 3.4 ± 5.2 years. Ten patients required valve surgery. Of 19 with severe AVVR at follow-up, 8 had severe AVVR at initial presentation and at follow-up (group B), whilst 11 had minimal AVVR at presentation but developed severe AVVR at their pre-Glenn echocardiogram (group C). Both group B and C at their initial echocardiogram had greater tenting height and indexed atrial area compared to Group A. Importantly 36% of Group C patients required valve surgery and 36% were symptomatic from AVVR. Multivariate analysis of group C identified initial echocardiogram absolute tenting height > 6mm (OR 9.5 (1.4-64.3), p=0.01) and indexed atrial area > 27cm/m2 (OR 7.3 (1.2-42.8), p=0.02) as independent predictors of subsequent severe AVVR. Severe AVVR in UAVSD is common and surgical intervention is frequent. Greater common valve tenting height and atrial size of non-regurgitant valves at the initial echocardiogram independently predicts subsequent severe AVVR. These indices may be useful in the management and decision process in UAVSD.

Background Atrioventricular valve regurgitation (AVVR) is the most common reason for reoperation after primum defect repair. Descriptions of pre-operative (pre-op) anatomical risk factors predictin...

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.

Atrioventricular valve regurgitation (AVVR) is the most common reason for reoperation after surgical repair of primum defects. Descriptions of pre-operative anatomical risk factors predicting significant post-operative AVVR are limited. This study utilized real-time three-dimensional echocardiography (RT-3DE) to examine pre-operative anatomical and functional characteristics of left atrioventricular valves in patients with primum defects for insights into post-operative valve failure. We reviewed 41 consecutive children (median age 3 years, range 2 months to 13 years) presenting to our institution for primum defect repair. Pre-operative two-dimensional echocardiography (2DE) was analyzed with 34 of 41 patients having RT-3DE available. At pre-operative and post-operative follow-up left AVVR (LAVVR) was graded by conventional 2DE. We assessed for specific pre-operative valvar or sub-valve risk factors, such as abnormally short chordae, commissural deformities and an eccentric cleft, using RT-3DE (n=34) and surgical reports (n=41). We compared the pre-operative valvar anatomy and function between patients who developed post-operative moderate LAVVR and those who did not. Thirty four percent had moderate pre-operative LAVVR. The mean post-operative follow-up was 23±16 months with 39% of patients having moderate LAVVR. In the cohort of 34 children with RT-3DE, we identified pre-operative valve or subvalve risk factors in 74% by RT-3DE and in 56% by surgical findings. Patients with moderate post-operative LAVVR were more likely to have significant pre-op LAVVR and abnormal RT-3DE findings than patients with mild post-operative LAVVR. There was no difference in the rate of abnormal surgical findings between these two groups. Moderate pre-operative LAVVR had a positive predictive value (PPV) for post-operative regurgitation of 77%. Abnormal RT-3DE findings had a negative predictive value of 100%. Combining these variables improved PPV marginally (83%). Surgical findings were lower than both of these values. In addition to moderate pre-operative LAVVR, valve and sub-valve abnormalities are risk factors for significant regurgitation after primum repair. RT-3DE is a sensitive tool to identify these anatomical risk factors. Early identification of these patients has the potential to facilitate surgical planning and procedure modification, and to improve the morbidity outcome of this difficult lesion.