BACKGROUND:Tricuspid regurgitation (TR) in hypoplastic left heart syndrome (HLHS) is associated with morbidity and mortality. TR mechanisms and the impact of tricuspid valve repair (TVR) are unclear. We examined HLHS TR mechanisms, TVR's impact on tricuspid valve (TV), and features of poor TVR durability.METHODS:We retrospectively compared 35 HLHS TVR cases and 35 age/stage-matched HLHS controls who do not undergo TVR. Pre-operative 3-dimensional echocardiography (3DE) assessed overall TV morphology (prolapse, normal, tethered), leaflet morphology, vena contracta area, and TR location. Two-dimensional echocardiography measured TV annulus diameter, RV fractional area change (RVFAC), sphericity, and TR grade at three time points (pre-op, early post-op, and latest follow-up).RESULTS:Pre-op, TVR group, and controls had no difference in age, RV function or shape, or TV dimension. TVR group most commonly had anterior leaflet prolapse followed by septal leaflet prolapse or tethering. TR jet arises centrally (63%) and anterior septally (26%). Posterior annuloplasty (69%), commissuroplasty (37%), and leaflet repair (37%) were surgical techniques commonly performed. At early post-op, TR grade and TV annulus decreased. At latest follow-up, TV annulus remained reduced; however, 50% had significant TR. 25% required TV reoperation. Larger vena contracta at TVR was associated with significant TR.CONCLUSION:HLHS patients undergoing TVR had more anterior leaflet prolapse and central TR. While TVR initially reduces annular size and TR grade, 50% redevelop significant TR despite maintained annular reduction. The association of greater TR severity prior to repair with post-op recurrence raises the consideration for earlier repair of TR in HLHS patients.

Right ventricular (RV) remodeling in hypoplastic left heart syndrome (HLHS) begins prenatally and continues through staged palliations. However, it is unclear if the most marked observed remodeling post-Norwood is secondary to cardiopulmonary bypass (CPB) exposure or if it is an adaptation intrinsic to the systemic RV. This study aims to determine the impact of CPB on RV remodeling in HLHS. Echocardiograms of HLHS survivors undergoing stage 1 Norwood (n = 26) or Hybrid (n = 20) were analyzed at pre- and post-stage 1, pre- and post-bidirectional cavo-pulmonary anastomosis (BCPA), and pre-Fontan. RV fractional area change (FAC), vector velocity imaging for longitudinal & derived circumferential deformation (global radial shortening (GRS) = peak radial displacement/end-diastolic diameter), and deformation ratio (longitudinal/ circumferential) were assessed. Both groups had similar age, clinical status and functional parameters pre-stage 1. No difference in RV size and sphericity at any stage between groups. RVFAC was normal (> 35%) throughout for both groups. Both Norwood and Hybrid patients had increased GRS (p = 0.0001) post-stage 1 and corresponding unchanged longitudinal strain, resulting in decreased deformation ratio (greater relative RV circumferential contraction), p = 0.0001. Deformation ratio remained decreased in both groups in subsequent stages. Irrespective of timing of the first CPB exposure, both Norwood and Hybrid patients underwent similar RV remodeling, with relative increase in circumferential to longitudinal contraction soon after stage 1 palliation. The observed RV remodeling in HLHS survivors were minimally impacted by CPB.

Adult patients with repaired congenital heart disease are presenting with previously unseen types of residual lesions and consequences of prior repair. Patients with d-transposition of the great arteries repaired with atrial switch operations are returning with dysrhythmias and atrioventricular valve disease requiring intervention. We present the challenging case of a young adult with a residual shunt identified on preoperative three-dimensional transthoracic echocardiography, the precise anatomy of which was only characterized intraoperatively.

BACKGROUND:Tricuspid regurgitation (TR) is an important risk factor for morbidity and mortality in hypoplastic left heart syndrome (HLHS), yet the evolution of tricuspid valve (TV) dysfunction in HLHS is poorly understood. This study sought to examine changes in TV function in HLHS between the first two stages of surgical palliation and to determine the mechanism of TR at the time of stage two surgery-bidirectional cavopulmonary anastomosis (BCPA). METHODS:We prospectively investigated 44 infants at two time points-prior to Norwood-Sano (T1 - median age 5.4 days) and prior to BCPA (T2 - median age 4.7 months) using two-dimensional (2DE) and three-dimensional echocardiography (3DE). Right ventricular (RV) size, function and shape was assessed with 2DE. Extracted spatial coordinates from 3DE were used to calculate TV leaflet and annular area, tethering and prolapse volumes, bending angle, and coaptation index. TR was graded qualitatively, and 2D and 3D vena contracta (VC) were measured. RESULTS:The cohort from T1 to T2 had increased indexed leaflet and annular area (P < .0001) and tethering volume (P < .0001), with no change in coaptation. Significant TR was present in 14 infants (32%) at T2 and was associated with greater leaflet (P = .02) and annular areas (P = .002) and greater prolapse volume (P = .008), but not tethering volume or reduced coaptation. At latest follow-up (median 23 months), 13 patients died or required transplantation. Only 3DE VC at T2 was associated with death or transplantation. CONCLUSIONS:The TV in HLHS adapts to interstage stressors (increased preload and afterload) by increasing leaflet size to maintain adequate leaflet coaptation. Significant TR at T2 was associated with greater leaflet size and prolapse. This may represent TV maladaptation from an excessive response in leaflet expansion to stressors.

Background: Single right ventricles (SRV) are postulated to be disadvantaged compared with single left ventricles (SLV). We compared the evolution of SRV versus SLV function during infancy using conventional measures and speckle-tracking echocardiography (STE). We hypothesized that the SRV is mechanically disadvantaged during early infancy.Methods: SRVs (n = 32) were compared with SLVs (n = 16) at the neonatal (presurgery) and pre-bidirectional cavopulmonary anastomosis (pre-BCPA) stages. Functional measures (fractional area change, indexed ventricular annular plane systolic excursion [iVAPSE], isovolumic acceleration [IVA], myocardial performance index, E and A velocities, tissue Doppler imaging annular velocities and STE-measured global longitudinal and circumferential strain, strain rate [SR], and early diastolic SR [EDSR]) were compared between SRV and SLV at each stage and between presurgery and pre-BCPA.Results: Compared with SLV, presurgery SRV had lower circumferential strain (-10.6% vs 16.5%; P =.0002) and EDSR (1.41%/sec vs 2.13%/sec; P =.001). Pre-BCPA SRV had decreased IVA (1.2 vs 2.1 m/sec(2); P =.006): longitudinal strain (-15.3% vs - 19.1%; P=.001), SR(-0.97%/sec vs -1.53%/sec; P=.0001), EDSR(1.5%/sec vs 2.1%/sec; P =.001); circumferential strain (-10.6% vs - 14.9%; P =.002), SR (-0.8%/sec vs -1.21%/sec; P =.0001), and EDSR (1.3%/sec vs 1.8%/sec; P =.009). SRV showed reduction of iVAPSE, IVA, s', e', a' velocities, longitudinal strain, SR, EDSR, and circumferential SR (P <.05) from presurgery to pre-BCPA, while circumferential strain was unchanged. SLV showed no significant change in these parameters during this interval.Conclusions: The progressive reduction in SRV longitudinal and circumferential function suggests that SRV may have a mechanical disadvantage from birth and progressive impairment with age.

Tricuspid regurgitation (TR) in infancy poses a surgical challenge. Both two- and three-dimensional echocardiography (3DE) can provide detailed information about the mechanism(s) of valve failure and insights into valve adaptation during follow-up. We report two patients who underwent tricuspid valve repair using Gore-Tex neochordae, repairs which were facilitated by and assessed with 3DE. Both infants had less than mild residual TR and no valve tethering at hospital discharge. Furthermore, follow-up 3DEs have helped to confirm valve competence, lack of tethering, and growth of the valve and valve apparatus.

Background: Tricuspid valve regurgitation (TR) is associated with morbidity and mortality in hypoplastic left heart syndrome (HLHS). Successful tricuspid valve repair (TVR) may improve outcomes, however 30-50% of patients develop significant post-operative TR, often requiring further surgery. We sought to determine pre-operative factors associated with development of TR after TVR in HLHS. Methods: Twenty-six HLHS patients requiring TVR for TR between 2005 and 2015 were assessed. 2D echocardiography (2DE) was used pre-op and late post-op (> 6 months) to assess TR grade, vena contracta (VC) size and annulus size. Patients were divided into those with ≤ mild TR (group A) and those with ≥ moderate TR (group B) at late post-op. Pre-op 3D echocardiography (3DE) was used to assess mechanism and location of TR. Surgical notes were reviewed for repair details. Results: Fourteen patients (54%) had ≤ mild TR (group A) and 12 (46%) had ≥ moderate TR (group B) at late post-op. TVR occurred most commonly with other surgical procedures (2 at Norwood, 8 at Glenn, and 7 at Fontan). On preop 3DE, 13 (52%) had central TR, 7 (28%) had central TR with extension into a commissure, 2 (8%) had anterior-septal TR, and 3 (12%) had mixed commissural TR. The primary mechanism was tethering in 14 (56%) and prolapse in 12 (44%). For surgical repair, 17 (65%) had posterior annuloplasty, 5 (19%) cleft closure, and 4 (16%) other. Types of surgical repair were similar between the 2 groups. Group A had smaller pre-op 2DE TV annulus Z-score (3.4 vs 5.0, P=0.013) but no difference in pre-op 2DE VC diameter, when compared to group B. Group A pre and post op-comparison showed reduced annulus Z-score (3.4 vs 0.8, P=0.01) and VC diameter (p=0.01), while Group B pre and post op annulus size and VC size showed no reduction. There was no difference between group A and B with respect to TR location or primary mechanism involved. Late post-op VC demonstrated moderate correlation with pre-op TV annulus size (P=0.005; R=0.54) but not with pre-op VC. Conclusion: TR after valve repair is common. Larger pre op annulus size is related to post-operative TR. Despite surgical repair aimed at reducing TV annulus size, failure to adequately reduce TV size appears to be a mechanism of persistent significant post-operative regurgitation in HLHS.

Background: Tricuspid regurgitation (TR) is associated with morbidity and mortality in hypoplastic left heart syndrome (HLHS). We have previously demonstrated that TR prior to stage 2 (S2) surgical palliation is related to tricuspid valve (TV) abnormalities, including dilated annulus and increased leaflet prolapse. Right ventricular (RV) dysfunction and mechanical dyssynchrony have been reported as causes for TR. Thus, we hypothesized that TR and abnormal TV parameters are associated with RV dysfunction. This study explored the relationship between TV and RV function using quantitative three dimensional echocardiography (3DE) measure of TV parameters and speckle tracking echocardiography (STE) of RV deformation, in HLHS with and without TR. Methods and Results: Forty-four HLHS patients with median age of 4.7 months (IQR 3.9-5.3) were prospectively recruited prior to S2 palliation. TV parameters assessed using 3DE, included vena contracta area (VCA), leaflet area, prolapse volume, tethering volume, annular bending angle and papillary muscle angle. RV systolic function was assessed by fractional area change and STE derived longitudinal and circumferential strain, strain rate and mechanical dyssynchrony index (MDI). The group was divided into those with (group A) and those without (group B) significant TR by qualitative assessment. RV functional parameters were compared between the two groups using Mann-Whitney signed rank test and Spearman correlation of TV leaflet and annulus area, prolapse and tethering volume, bending angle and VCA, to longitudinal and circumferential strain, strain rate and MDI were performed. Fourteen patients (32%) had moderate or greater TR (group A). RV function parameters were not different between group A and group B. There was no correlation between all 3DE TV parameters and STE RV deformation parameters. Conclusion: Contrary to our hypothesis, HLHS RV systolic dysfunction and mechanical dyssynchrony do not play a significant role in early TR. This finding suggests the mechanisms of early TR in HLHS are likely to be secondary to valve and leaflet differences.

Background: A recent study using two-dimensional echocardiogram (2DE) reported a predominance of antero-septal commissural regurgitation in patients (70%) undergoing tricuspid valve repair (TVR) in hypoplastic left heart syndrome (HLHS). Three-dimensional echocardiography (3DE) studies on assessment of TV morphology reports a high incidence of error in the identification of TV leaflets in conventional 2DE planes. This study aims to assess the pre-operative 2DE assessment of TR jet location compared to the reference standard of 3DE color datasets in HLHS. Methods: Twenty-five HLHS patients requiring TVR for TR between 2005 and 2015 were assessed using 2DE and 3DE to assess TR grade, 3DE vena contracta (VC) area, TV annulus diameter (indexed to body surface area, iTV) and primary TR jet location. The 2DE observer was blinded to the findings on 3DE. We compared the 2DE findings with 3DE using t-test and Pearson correlation. Surgical notes were reviewed for details of procedures performed. Results: Indexed 2DE iTV was no different to 3DE (61mm/m 2 ± 4 vs. 56mm/m 2 ± 4, p=0.34). 2DE qualitative TR grade correlated with 3DE VC area (r=0.69, p Conclusions: 2DE was accurate in the assessment of the degree of TR and TV annular size, but in most patients incorrectly identified the primary regurgitation location in the antero-septal region instead of other location. This may have implications for planning of the primary surgical procedure in HLHS TV repair based on 2DE findings.

Objective: Significant atrioventricular valve regurgitation (AVVR) increases mortality in patients with unbalanced atrioventricular septal defects (uAVSDs) and a single ventricle. We tested the hypothesis that abnormal leaflet tethering is associated with progressive AVVR in patients with a single ventricle with uAVSD.Methods: We retrospectively reviewed the initial presentation and prebidirectional cavopulmonary anastamosis echocardiograms of 46 consecutive patients with uAVSD with single ventricle palliation. AVVR was graded as moderate to severe if the sum of vena contracta width to dominant valve annulus ratio was >= 0.33. We measured tenting height, annular to leaflet angle and annular diameter, indexed to patient size where appropriate. Multivariate analysis of variables to predict progressive AVVR was performed.Results: At follow-up of 3.3 +/- 2.4 years, 24 patients had mild AVVR (Group A) and 22 had moderate to severe AVVR. Overall mortality was 6%, whereas 10 had valve repair/replacement surgery. Of 22 patients with severe AVVR at follow-up, 9 had severe AVVR at initial presentation (Group B), whereas 13 had mild AVVR at presentation but developed severe AVVR at their prebidirectional cavopulmonary anastamosis echocardiogram (Group C). Group A patients had a smaller tenting height at initial presentation compared with patients in Group B and Group C, and also had early progressive reduction of indexed tenting height (P <. 01). An absolute tenting height > 6 mm (odds ratio, 6.6; 95% confidence interval, 1.1-39.0; P =.03) at the initial echocardiogram was identified as an independent predictor of subsequent severe AVVR.Conclusions: Early leaflet tethering is predictive of subsequent AVVR in patients with a single ventricle with uAVSD. Patients with competent AVV had progressive reduction in the degree of leaflet tethering, whereas patients with AVVR did not. This may represent an important adaptive process to maintain valve competency in uAVSD. (J Thorac Cardiovasc Surg 2014; 148: 2580-8)

Echocardiography has developed as an imaging technology over 60 years to become the mainstay for investigating heart disease, providing invaluable structural and functional information. In the last 20 years, 3-dimensional echocardiography (3DE) has emerged as an adjunct to 2-dimensional echocardiography in adult and congenital heart disease. Early work with 3-dimensional imaging of the mitral valve describing normal annular shape and function significantly changed the understanding of mitral valve dynamics. Further work led to our current understanding of the mitral valve working as a unit, with all components vital to its normal function. With improving technology and ease of use, similar 3DE techniques have been used in congenital heart disease to study the unique anatomy and function of atrioventricular (AV) valves, specifically the tricuspid valve in hypoplastic left heart syndrome, and the left AV valve in atrioventricular septal defects. This paper describes the role of 3DE in assessing AV valve function in normal valves, and in congenital heart disease.

Different surgical techniques for complete atrioventricular septal defect (CAVSD) repair have been described, with the double-patch technique being most frequently employed. More recently a newer technique using a modified single-patch repair has been advocated. We hypothesized that the modified single-patch technique would result in an increased incidence of the two major post-repair comorbidities, namely, distortion of the left AV valve (LAVV) leaflets and narrowing of the left-ventricular outflow tract (LVOT). We studied 14 patients with CAVSD who underwent either traditional double-patch technique [group 1 (n = 7)] or modified single-patch technique [group 2 (n = 7)]. Preoperative and immediate postoperative two-dimensional (2D) echocardiograms, as well as follow-up 2D and three-dimensional (3D) studies, were reviewed. For group 1, the median age at repair was 4.1 months with a median duration from surgical repair and last echocardiogram of 44 months. For group 2, the median age at repair was 3 months with a median duration from surgical repair and last echocardiogram of 28 months. The two groups had similar demographics and ventricular septal defect size before surgery. For the LAVV, no significant difference was observed with respect to LAVV annulus size, tenting height, and the size of the vena contracta. Furthermore, there was no significant difference in the 2D echocardiographic areas and volumes of the LVOT between pre-repair and immediate post-repair studies for both groups. At the last evaluation, although there had been growth of the LVOT in both groups, no significant difference between areas and volumes were observed. Areas of the LVOT measured by 3D echocardiography on the final study showed no significant statistical difference between both groups. There was good correlation of the areas measured by 2D and 3D echocardiography within each group. In this small group, modified single-patch technique does not appear to tether the LAVV or promote an increase in regurgitation. In the short term, LVOT growth is unaffected, and the repair does not promote LVOT obstruction. 3D echocardiography is useful for area measurements of the LVOT and showed good correlation with areas measured by assumption of the LVOT shape as determined using 2D techniques.

Background: Single ventricle (SV) exercise performance is impaired and limited by reduced ventricular preload reserve. The atrium modulates ventricular filling, and enhancement of atrial compliance can increase cardiac performance. We aimed to study atrial mechanics in SV hearts across staged surgical palliation compared with healthy children by using novel speckle-tracking echocardiography techniques.Methods: A cross-sectional study of 81 patients with SV (1 day to 6.5 years) at 4 stages of surgical palliation (presurgery, 22; prebidirectional cavopulmonary anastomosis, 23; pre-Fontan, 22; post-Fontan, 14). The dominant atrium was assessed with speckle-tracking echocardiography for active (epsilon(act)), conduit (epsilon(con)), and reservoir (epsilon(res)) strain; strain rate (SR); and epsilon(act)/epsilon(res) ratio before each stage of surgical palliation. Findings were compared with the left atrium of 51 healthy children (1 day to 5.5 years).Results: Single ventricle atrial size was increased (P < .01), and atrial eres was decreased (P < .01) compared with healthy controls. SV atrial e(con) (P < .01) and SRcon (P < .0001) was decreased, increased epsilon(act) persisted (P < .05), and epsilon(act)/epsilon(res) was increased (P < .001) between surgical stages. Although the expected maturational trend of increasing epsilon(con), decreasing e(act), and epsilon(act)/epsilon(res) occurred in SV, they lagged behind healthy maturational changes (P < .0001).Conclusion: Single ventricle atrium is dilated, has deceased compliance, decreased early diastolic emptying, and increased reliance on active atrial contraction for ventricular filling. This deviates from normal early childhood maturational changes and appears to parallel those of an atrium facing early ventricular diastolic dysfunction.

BACKGROUND:Long-term survival after atrioventricular septal defect repair is excellent; however, postoperative left atrioventricular valve regurgitation affects morbidity and quality of life. Left atrioventricular valve regurgitation is the most common reason for reoperation after repair and it is critical that clinicians recognize pathologic mechanisms pre-repair. METHODS:In this single-center experience, we identified a pattern of left atrioventricular valve abnormality in 5 cases presenting for routine surgical repair between 1 month and 24 years of age. We reviewed two-dimensional and real-time three-dimensional echocardiographic and surgical findings to assess for specific valvar or sub-valve abnormalities, including short chordae, commissural deformities, and an eccentric zone of apposition. Two-dimensional echocardiography was used to assess the degree of preoperative and postoperative left atrioventricular valve regurgitation. RESULTS:Abnormal features identified included short, thickened chordae, poorly formed superior-mural commissure, and an eccentric zone of apposition. At surgical repair, 2 patients had limited closure of the zone of apposition, as part of a complete repair, and developed only mild left atrioventricular valve regurgitation in short-term follow-up. Two further patients had attempted complete closure of the zone of apposition with moderate postoperative regurgitation ultimately necessitating left atrioventricular valve replacement. CONCLUSIONS:This uncommon form of atrioventricular septal defect is identifiable with echocardiography and may be associated with significant postoperative regurgitation if the zone of apposition is completely sutured at time of repair. Limited closure of the zone of apposition may improve postoperative regurgitation.

We present a case of an infant prenatally diagnosed with bilateral outflow-tract obstruction and severe aortic regurgitation who underwent cardiac transplantation at 45 days of life. Aortico-left ventricular tunnel was subsequently diagnosed on pathologic examination of the explant heart. Aortico-left ventricular tunnel is a rare congenital cardiac malformation and can remain undiagnosed if the clinician has a low level of suspicion. Aortico-left ventricular tunnel should be considered in any fetus or newborn with aortic regurgitation.

Background: Differences in single right ventricle (SRV) and single left ventricles (SLV) function are poorly described, although myocardial dysfunction is an important risk factor for morbidity and mortality. The aims of this study were to compare function between patients with SRVs and those with SLVs using newer echocardiographic techniques and to determine differences across staged palliation.Methods: In this cross-sectional study comparing 30 patients with SRVs and 30 with SLVs of similar ages (2.5 61.7 vs 2.6 +/- 1.6 years), patients were matched for surgical stage (20 pre-bidirectional cavopulmonary anastomosis, 20 pre-Fontan, and 20 post-Fontan patients). Circumferential and longitudinal strain, strain rate (SR), early diastolic SR, postsystolic strain index, and myocardial dyssynchrony index were measured. Comparisons between SRV and SLV parameters were made as a whole group and by subanalysis at each surgical stage.Results: Patients with SRVs had reduced systolic SRs (circumferential: -1.0%/sec vs -1.2%/sec, P = .01; longitudinal: 1.1%/sec vs 1.3%/sec, P = .002), reduced early diastolic SRs (circumferential: 1.4%/sec vs 1.9%/sec, P = .03; longitudinal: 1.6%/sec vs 2.2%/sec, P = .001), and increased circumferential postsystolic strain indexes (8% vs 0%, P < .0001). Subanalysis at each surgical stage showed that the greatest disparity in systolic parameters occurred before bidirectional cavopulmonary anastomosis (longitudinal SR, P = .009; postsystolic strain index, P = .005) and that parity was regained after the Fontan procedure, while traditional diastolic parameters (E velocity, P = .004; E/E0 ratio, P = .0003) were reduced in patients with SRVs after the Fontan procedure.Conclusions: The SRV has reduced contractility and diastolic function compared with the SLV. Ventricular systolic performance in patients with SRVs was poorest before bidirectional cavopulmonary anastomosis, while differences in diastolic function were more prominent after Fontan completion. (J Am Soc Echocardiogr 2012;25:1222-30.)

Atrioventricular septal defects comprise a disease spectrum characterized by deficient atrioventricular septation, with several common features seen in all affected hearts and variability in atrioventricular valve morphology and interatrial and interventricular communications. Atrioventricular septal defects are among the more common defects encountered by pediatric cardiologists and echocardiographers. Despite advances in understanding, standard two-dimensional echocardiography may not be the optimal method for the morphologic and functional evaluation of this lesion, particularly malformations of the atrioventricular valve(s). In this review, the authors summarize the role of three-dimensional echocardiography in the diagnostic evaluation of atrioventricular septal defects.