Left Atrioventricular Valve Regurgitation Research Articles

Predictors of post-operative left atrioventricular valve regurgitation in pediatric patients with complete atrioventricular canal defects.

In infants with complete atrioventricular canal (CAVC) defects, post-operative left atrioventricular valve regurgitation (LAVVR) is a known major cause of morbidity and mortality and a common indication for re-operation. However, there is scarce data to identify risk factors for poor outcomes. Our study aims to find echocardiographic characteristics that predict post-operative LAVVR at discharge and 1-year follow-up. Retrospective cohort study of patients with initial CAVC repair at our hospital who were followed for 1 year between 2013 and 2022. Patients with major co-morbid conditions were excluded. Serial echocardiograms were reviewed. Anatomic details, quantitative and qualitative measure of LAVVR including the number of regurgitant jets, regurgitant jet length and vena contracta width, and ventricular function were collected. The time points measured include pre-operative transthoracic echocardiogram (TTE), post-operative transesophageal echocardiogram (PO-TEE), routine protocol based post-operative day 1 (POD1) TTE, discharge TTE and 1-year post-operative (1yPO) TTE. Paired t-tests, chi-square analysis, and linear regression analysis were performed comparing measured variables to LAVVR outcomes. Fifty-two patients were included; 92% had Trisomy 21. The majority were classified as Rastelli A (71%), others Rastelli C (29%). Only two patients had moderate or greater LAVVR pre-operatively. The mean age at repair was 125 ± 44 days. Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year after backward stepwise regression. Of those with<moderate LAVVR on PO-TEE, 20% had worsening to≥moderate at discharge, but only 9% remained that way at 1 year. Of those with≥moderate LAVVR on PO-TEE, 40% improved to<moderate by 1 year. Two patients who worsened at 1 year, both secondary to likely cleft suture dehiscence. Only one patient required reoperation in the immediate post-operative period secondary to severe LAVVR due to suture dehiscence. Routine protocol-based POD1 echo did not have any association with altered outcomes. Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year. A significant percentage (40%) of patient with≥moderate LAVVR on PO-TEE improved to<moderate by 1 year. Furthermore, routine protocol-based POD1 echo did not have any association with altered outcomes.

Repair of the complete atrioventricular septal defect-impact of postoperative moderate or more regurgitation.

To study the risk factors for mortality, moderate or more left atrioventricular valve regurgitation (LAVVR) and reoperation after the surgical repair of complete atrioventricular septal defect (cAVSD) in a single centre. The current study is a retrospective review of patients who underwent surgical repair of cAVSD between 2000 and 2021. Patients with unbalanced ventricles not amenable to biventricular repair, double outlet right ventricle and malpositioned great arteries were excluded. The clinical predictors of outcome for end points were analysed with univariate and multivariable Cox regression analysis or Fine-Gray modelling for competing risks. Time-dependent end points were estimated using the Kaplan-Meier curve analysis and cumulative incidence curves. The median follow-up time was 2.3 years. Among 220 consecutive patients were 10 (4.6%) operative and 21 late mortalities (9.6%). A total of 26 patients were identified to have immediate postoperative moderate or more regurgitation and 10 of them ultimately died. By multivariable analysis prematurity and having more than moderate regurgitation immediately after the operation were identified as predictors of overall mortality (P = 0.003, P = 0.012). Five- and ten-year survival rates were lower for patients with immediate postoperative moderate or more LAVVR {51.9% [confidence interval (CI): 27.5-71.7%]} when compared to patients without moderate or more regurgitation [93.2% (CI: 87.1-96.4%) and 91.3% (CI: 83.6-95.5%)]. The patients who undergo cAVSD repair remain subjected to a heavy burden of disease related to postoperative residual LAVVR. Immediate postoperative moderate or more LAVVR contributes significantly to overall mortality. Whether a second run of bypass can decrease this observed mortality should be investigated.

Cleft closure and other predictors of contemporary outcomes after atrioventricular canal repair in patients with parachute left atrioventricular valve.

Parachute left atrioventricular valve (LAVV) complicates atrioventricular septal defect (AVSD) repair. We evaluate outcomes of AVSD patients with parachute LAVV and identify risk factors for adverse outcomes. We evaluated all patients undergoing repair of AVSD with parachute LAVV from 2012 to 2021. The primary outcome was a composite of time-to-death, LAVV reintervention and development of greater than or equal to moderate LAVV dysfunction (greater than or equal to moderate LAVV stenosis and/or LAVV regurgitation). Event-free survival for the composite outcome was estimated using Kaplan-Meier methodology and competing risks analysis. Cox proportional hazards regression was used to identify predictors of the primary outcome. A total of 36 patients were included with a median age at repair of 4 months (interquartile range 2.3-5.5 months). Over a median follow-up of 2.6 years (interquartile range 1.0-5.6 years), 6 (17%) patients underwent LAVV reintervention. All 6 patients who underwent LAVV reintervention had right-dominant AVSD. Sixteen patients (44%) met the composite outcome, and all did so within 2 years of initial repair. Transitional AVSD (versus complete), prior single-ventricle palliation, leaving the cleft completely open and greater than or equal to moderate preoperative LAVV regurgitation were associated with a higher risk of LAVV reintervention in univariate analysis. In multivariate analysis, leaving the cleft completely open was associated with the composite outcome. Repair of AVSD with parachute LAVV remains a challenge with a significant burden of LAVV reintervention and dysfunction in medium-term follow-up. Unbalanced, right-dominant AVSDs are at higher risk for LAVV reintervention. Leaving the cleft completely open might independently predict poor overall outcomes and should be avoided when possible. IRB-P00041642.

Early and mid-term results of surgical treatment of atrioventricular septal defect; analysis of risk factors for adverse outcomes: left atrioventricular valve regurgitation

Background Advances in the perioperative management and optimization of the timing of primary repair have gradually improved the early clinical outcome of surgical correction of atrioventricular septal defects (AVSDs). However, the late outcome still remains compromised by the substantial risk of reoperation, of which mainly the development of left ventricular outflow tract obstruction (LVOTO) and left atrioventricular valve (LAVV) dysfunction challenge the long-term survival and morbidity of these children. In particular, LAVV regurgitation (LAVVR) is responsible for a reoperation rate between 5 and 19%, both for complete AVSD (cAVSD) and partial AVSD (PAVSD) children. Results of surgical correction of AVSDs) have improved over the last decades; however, the need for reoperation after primary AVSD repair remains a major concern. We conducted this study to analyze risk factors leading to LAVVR in the early and mid-term follow-up postsurgical repair of AVSD. Patients and methods All patients who underwent biventricular repair for AVSD at our institute, in the time between Jan 2015 and Jan 2021, meeting the inclusion criteria and none of the exclusion criteria were enrolled in the study. Preoperative, operative and postoperative data were collected from the patients’ medical file. Last follow-up Echoes were reviewed. Results Patients who had preoperative and early postoperative more than moderate LAVVR are more likely to develop LAVVR in the late follow-up and also have a more risk to need a reoperation in their midterm follow-up. Conclusions Patients who had preoperative and early postoperative more than mod LAVVR are more likely to develop LAVVR in the late follow-up and also more likely to need reoperation.

Staged repair for complete atrioventricular septal defect in patients weighing less than 4.0 kg

ObjectiveThis study compared the mortality, left atrioventricular valve–related reoperation, and left atrioventricular valve competence in symptomatic neonates and small infants who underwent staged repair incorporating pulmonary artery banding or primary repair for complete atrioventricular septal defect. MethodsPatients weighing less than 4.0 kg at the time of undergoing staged (n = 37) or primary (n = 23) repair for balanced complete atrioventricular septal defect between 1999 and 2022 were reviewed. The mean follow-up period was 9.1 years. Freedom from moderate or greater left atrioventricular valve regurgitation was estimated with the Kaplan–Meier method. ResultsThe staged group included smaller children (median weight, 2.9 vs 3.7 kg) and a higher proportion of neonates (41% vs 4%). All patients in the staged group survived pulmonary artery banding and underwent intracardiac repair (median weight, 6.8 kg). After pulmonary artery banding, the severity of left atrioventricular valve regurgitation improved in 10 of 12 patients (83%) without left atrioventricular valve anomaly who had mild or greater left atrioventricular valve regurgitation and a left atrioventricular valve Z score greater than 0. Although survival and freedom from left atrioventricular valve–related reoperation at 15 years (P = .195 and .602, respectively) were comparable between the groups, freedom from moderate or greater left atrioventricular valve regurgitation at 15 years was higher in the staged group (P = .026). ConclusionsCompared with primary repair, staged repair for complete atrioventricular septal defect in children weighing less than 4.0 kg resulted in comparable survival and reoperation rates and better left atrioventricular valve competence. Pulmonary artery banding may mitigate secondary left atrioventricular valve regurgitation unless a structural valve abnormality exists. Selective deferred intracardiac repair beyond the neonatal and small-infancy period may still play an important role in low-weight patients.

The Impact of Postoperative Left Atrioventricular Valve Regurgitation on Outcomes of Complete AVSD Repair

Left atrioventricular valve (LAVV) regurgitation is the major cause of morbidity and mortality following repair of complete atrioventricular septal defects (cAVSD). However, the impact of different postoperative degrees of LAVV regurgitation (LAVVR), and thus indication for early reintervention, remains unclear.

Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability

ObjectiveAcute outcomes after atrioventricular canal defects (AVCD) surgery in the current era are excellent; yet despite surgical advances, ∼15% of patients require future left atrioventricular valve (LAVV) repair. Among patients with AVC who undergo LAVV repair after primary AVC surgery, we sought to characterize the durability of these repairs. Specifically, we aimed to determine predictors for reintervention following an LAVV repair in patients with repaired AVCD, with a focus on postoperative transesophageal echocardiography (TEE). MethodsWe reviewed all patients undergoing LAVV repair (after a primary AVCD surgery) at Boston Children's Hospital between 2010 and 2020. Competing risk analysis was performed to evaluate cumulative incidence of LAVV reinterventions. Predictors of LAVV reintervention were evaluated using multivariable Cox regression. ResultsA total of 137 LAVV repairs following primary AVCD surgery were performed in 113 patients. Median age and weight at LAVV repair were 25 months (interquartile range, 12-76 months) and 11.1 kg (interquartile range, 7.8-19.4 kg). Original anatomy was complete AVCD in 87 (63%), transitional AVCD in 27 (20%), and partial AVCD in 23 (17%) cases. Over a median follow-up of 12 months (interquartile range, 1.3 months-4 years), 47 (34%) of the LAVV repairs required LAVV reintervention. Reinterventions included a total of 27 LAVV re-repairs and 20 LAVV replacements. In multivariable analysis, age at LAVV repair younger than 72 months, partial AVCD anatomy, left ventricle dysfunction, mean LAVV stenosis gradient ≥5 mm Hg, and multiple jets of regurgitation on postoperative LAVV repair TEE were associated with LAVV reintervention. Grade of LAVV regurgitation on postoperative TEE was not an independent risk factor, but reintervention rates were high when residual LAVV stenosis gradient was ≥5 mm Hg and residual mild LAVV regurgitation was present on postoperative TEE (47%) and even higher when residual LAVV stenosis gradient was ≥5 mm Hg and LAVV regurgitation was greater than mild (73%). ConclusionsReintervention rates remain high for LAVV repairs that occur after primary AVCD surgery, particularly for patients with LAVV stenosis gradient ≥5 mm Hg and mild or greater LAVV regurgitation on postoperative TEE.

Abstract 9841: Outcomes Following Biventricular Repair of Atrioventricular Septal Defect With a Single Left Ventricular Papillary Muscle

Introduction: In patients with atrioventricular septal defect (AVSD) with single papillary muscle attachments in the left ventricle (LV), the left AV valve (LAVV) is at risk for suboptimal repair. This study aimed to evaluate outcomes following repair of AVSD with single LV papillary muscle attachments and to identify preoperative predictors of postoperative LAVV outcomes. Methods: All patients with AVSD and anatomically or functionally single LV papillary muscle who underwent biventricular repair at our institution from 07/01/2000 - 07/01/2020 were retrospectively reviewed. Univariate analysis was used to identify associations between patient demographics, preoperative echocardiographic measures, and surgical characteristics with the degree of LAVV stenosis or regurgitation postoperatively and the need for reintervention on the LAVV. Results: Of 38 patients included, nearly all (97%) were repaired with no or partial closure of the LAVV cleft. Freedom from LAVV reintervention was 91% and 84% at 1 and 3 years. Five patients (13%) required reintervention on the LAVV with a median time to reintervention of 3.3 months post-AVSD repair; indication was LAVV regurgitation for four of the five patients. Preoperative LAVV regurgitation &gt; mild was associated with an increased risk for LAVV reintervention (p= 0.02). At discharge, 63% of patients had &gt; mild LAVV regurgitation and 5% had &gt; mild LAVV stenosis. At most recent follow-up, 32% had &gt; mild LAVV regurgitation and 12% had &gt; mild LAVV stenosis. Patients with younger age at repair and preoperative AV valve index &lt;0.67 had significantly increased risk of &gt; mild LAVV regurgitation at discharge (p=0.01 and p=0.04, respectively), however this did not persist at most recent follow-up. More pronounced unbalance of the AVSD was not associated with postoperative LAVV stenosis. Conclusions: Despite concern for repair in patients with AVSD and single papillary muscle attachments, surgical outcomes are favorable with low risk for reintervention. Risk of LAVV stenosis is low when repair of LAVV cleft is modified. Preoperative LAVV regurgitation, younger age at repair, and more pronounced unbalance of the AVSD predicted early postoperative LAVV regurgitation, with improvement over time.

Abstract 12837: A Novel Index to Predict Post-Operative Left Atrioventricular Valve Morbidity in Atrioventricular Septal Defects

Introduction: Morbidity of the left atrioventricular valve (LAVV) remains the leading indication for reoperation on atrioventricular septal defects (AVSDs). While qualitative 2D and quantitative 3D risk factors have been identified, no quantitative 2D measurement yet exists to predict poor postoperative outcomes. The aim of this study was to evaluate objective measurements of the LAVV and subvalvar apparatus in relation to postoperative outcomes. Methods: A retrospective review was performed on 2D echocardiograms of patients undergoing surgical repair of an AVSD from January 1, 2014 to December 31, 2021. Demographic and perioperative data were collected from the electronic medical record. The hingepoint-to-hingepoint length of the superior, mural and inferior bridging leaflets in early diastole, as well as LAVV chordal length at end-diastole were measured and indexed to body surface area. A poor outcome was defined by significant LAVV regurgitation (moderate or greater), LAVV stenosis (5mmHg or greater), need for second cardiopulmonary bypass run, postoperative ECMO, reoperation, or death. LAVV measurements were compared to composite outcome by Wilcoxon rank sum test. Results: Of 120 patients with adequate perioperative imaging, 47 (39.2%) had a poor outcome. Indexed LAVV chordal length was significantly different between those who experienced a poor outcome and those who did not, with shorter chordal length predictive of a poor outcome. Interestingly, mural leaflet size was not associated with a poor outcome. Conclusions: Indexed LAVV chordal length is a novel measurement shown to predict a poor postoperative outcome in AVSD. Additional analysis is necessary to assess whether short chords are more predictive of LAVV regurgitation or stenosis, and to determine a numeric cut-off of indexed chordal length for predicting poor outcome.

Middle to long-term outcomes of surgical repair for atrioventricular septal defect: a single-center study.

The exact incidence and predictors of mortality and left atrioventricular valve (LAVV) re-operation in congenital atrioventricular septal defect (AVSD) repair are still unclear. This study analyzed the middle to long-term outcomes of surgical repair for AVSD. A total of 150 patients (69 males and 81 females) who underwent AVSD repair at Children's Hospital of Fudan University from January 2013 to December 2021 were divided into complete defect group (C-group, 67 cases), transitional defect group (T-group, 26 cases), and partial defect group (P-group, 57 cases). Outcomes during the peri-operative and 10-year follow-up periods were evaluated. The total mortality was 5.33% (8/150), including seven early deaths (10.4%) and no late deaths in the C-group, no early deaths (0%) and one late death (1.8%) in the P-group, and no early or late deaths in the T-group. Up to the last follow-up, severe LAVV regurgitation had occurred in 27 patients, including 16 in the C-group, four in the T-group, and seven in the P-group. In total, 12 (12/150, 8.0%) patients received LAVV re-operation, including seven in the C-group, three in the T-group, and two in the P-group. Cox regression analysis showed that pre-operative severe pulmonary hypertension (P=0.006) and severe LAVV regurgitation within 24 hours after the first surgery (P=0.023) were independent risk factors for mortality. ≥ Moderate LAVV regurgitation within the first 24 hours after surgery (P=0.014) was an independent risk factor for LAVV re-operation. Complete AVSD repair increased the risk of early death, severe LAVV regurgitation and re-operation. Pre-operative severe pulmonary hypertension and residual severe LAVV regurgitation indicated high risk for mortality. ≥ Moderate LAVV regurgitation within 24 hours after the first surgery predicted a high probability of LAVV re-operation.

Modified single-patch repair for atrioventricular septal defects results in good functional outcomes in the absence of deep ventricular septal defects

ObjectivesWe compared 2-patch repair (TP) with modified single-patch repair (MSP) for complete atrioventricular septal defects and evaluated their effect on the left atrioventricular valve (LAVV) competence. We also identified risk factors for unfavorable functional outcomes. MethodsThis retrospective study included 118 patients with complete atrioventricular septal defects who underwent intracardiac repair from 1998 to 2020 (MSP: 69; TP: 49). The median follow-up period was 10.4 years. The functional outcome of freedom from moderate or greater LAVV regurgitation (LAVVR) was estimated using the Kaplan–Meier method. ResultsThe hospital mortality was 1.7% (2/118) and late mortality was 0.8% (1/118). Eight patients required LAVV-related reoperation (MSP: 4; TP: 4) and none required left ventricular outflow tract-related reoperation. In the MSP group without LAVV anomaly, the receiver operating characteristic curve analysis revealed that the ventricular septal defect (VSD) depth was strongly associated with moderate or greater postoperative LAVVR, with the best cutoff at 10.9 mm. When stratified according to the combination of intracardiac repair type and VSD depth, the MSP-deep VSD (VSD depth >11 mm) group showed the worst LAVV competence among the 4 groups (P = .002). According to multivariate analysis, weight <4.0 kg, LAVV anomaly, and moderate or greater preoperative LAVVR were independent risk factors for moderate or greater postoperative LAVVR, whereas MSP was not a risk factor. ConclusionsPostoperative LAVVR remains an obstacle to improved functional outcomes. MSP provides LAVV competence similar to TP unless deep VSD is present. The surgical approach should be selected on the basis of anatomical variations, specifically VSD depth.

A New-Onset Mass in the Right-Sided Atrium in a Child After Cardiac Surgery: Unravelling the Diagnosis

A 2-year-old girl (weight: 9 kg, height: 82 cm) underwent an intracardiac repair. Preoperative transthoracic echocardiography revealed situs solitus, levocardia, left atrial isomerism, common atrium due to a large atrial septal defect, absent coronary sinus, 2 separate atrioventricular valves with a trivial left atrioventricular valve regurgitation due to a cleft in the anterior leaflet, and a dilated right ventricle with flattening of the interventricular septum due to volume overload (Fig. 1).

Surgical Outcomes in Children With Partial and Transitional Atrioventricular Septal Defect.

This study aimed to evaluate the long-term outcomes of partial and transitional atrioventricular septal defect repair, focusing on left atrioventricular valve reoperation. We conducted a retrospective review of 104 children who underwent partial or transitional atrioventricular septal defect repair between August 1983 and January 2021. The major outcomes included patient survival and left atrioventricular valve reoperation. Median age at repair was 4.0 (0.1-17.0) years, with 17 patients being infants (age ≤ 1 year; 16.3%). All but eight patients (92.3%) underwent left atrioventricular valve cleft closure. After initial repair, there were 18 cases of moderate-to-severe left atrioventricular valve regurgitation (17.3%). Three in-hospital deaths (2.9%) and four late deaths (3.8%) occurred. At follow-up (median 14.3 years), actuarial survival was 95.1% and 93.0% at 1 and 20 years, respectively, and 16 patients (15.4%) had undergone a total of 19 left atrioventricular valve reoperations. Initial repair performed during infancy was associated with significantly higher mortality than a repair performed after infancy (35.3% vs 1.5%, P < .01, hazard ratio = 26.4). On multivariable analysis, repair during infancy was associated with mortality (P < .01, hazard ratio = 27.4, 95% confidence interval = 2.7-283). Partial or no cleft closure of left atrioventricular valve (P = .03, hazard ratio = 4.7, 95% confidence interval = 1.2-18.8) and moderate-to-severe left atrioventricular valve regurgitation after repair (P < .01, hazard ratio = 9.9, 95% confidence interval = 3.0-32.2) were associated with left atrioventricular valve reoperation. Partial and transitional atrioventricular septal defect repair outcomes were generally satisfactory. However, repair during infancy had worse survival outcomes, and moderate-to-severe left atrioventricular valve regurgitation after a repair was associated with future left atrioventricular valve reoperation.

Reoperations After Repair for Atrioventricular Septal Defects: >25 Years Experience at a Single Center

Our aim was to evaluate the total burden of reoperations after previous repair for atrioventricular septal defects, including long-term survival and identify risk factors for reoperation. All patients with surgical correction for atrioventricular septal defect (AVSD) 1993- 2020 underwent a follow-up in October 2020. Clinical data were obtained by retrospective review and evaluated with Kaplan-Meier and competing risk analysis. Of 477 patients who underwent initial repair, 53 patients (11.1%) underwent a total of 82 reoperations. The perioperative mortality at reoperation was 3.8% (2/53). There were no late deaths (0/51) during follow-up. In patients requiring reoperation for left atrioventricular valve regurgitation, a re-repair was performed in 90% (26/29) at first attempt. Estimated overall survival was 96.2 ± 2.6% (95% CI 91.2-100) in the Any reoperation group and 96.7 ± 0.9% (95% CI 94.9-98.5) in the No reoperation group at 20 years (P=0.80). The cumulative incidence function of Any reoperation (with death as competing risk) was 13.0% (95% CI 9.4-16.5) at 20 years. Independent risk factors for Any reoperation included severe mitral regurgitation after primary repair (HR 40.7; 95% CI 14.9-111; P < 0.001). The risk of perioperative mortality in AVSD patients undergoing reoperation was low in the present study. Long-term survival was very good and not significantly different when compared to patients who did not need reoperation. Re-repair for left atrioventricular valve regurgitation was possible in most cases and showed long-term durability. Our data suggest that reoperations after primary repair of AVSD have very good long-term outcomes when performed at a high-volume pediatric cardiac surgery center.

Single-Stage Surgical Management of Atrioventricular Septal Defects with Coarctation of the Aorta.

Surgical options for coarctation of aorta (CoA) with atrioventricular septal defect (AVSD) include single-stage repair vs. staged approach with neonatal CoA repair and delayed AVSD repair. The durability of left atrioventricular valve (LAVV) function after neonatal repair is questioned, and the optimal approach remains controversial. Eighteen CoA-AVSD patients who underwent single-stage repair 2005–2015 by a single surgeon were retrospectively analyzed. Fifteen patients had complete and three had partial AVSD. Birth weight was 3.19 kg (2.17–4.08). Age at surgery was 16 days (6–127). One- and ten-year survival were 80% and 69%. Freedom from reintervention was 60% and 40% at one and ten-year respectively. Reinterventions included relief of left ventricular outflow tract obstruction (LVOTO) (n = 4), repair of cleft LAVV (n = 3), and LAVV and aortic valve replacement (n = 1). Freedom from LAVV reintervention was 85.6% and 66% at 1 and 10 years respectively. There were four deaths: two post-operative and two following hospital discharge. Mortality was due to sepsis in three patients, and heart failure related to LVOTO and LAVV insufficiency in one. At 68-month (0.6–144) follow-up the majority had mild or less LAVV regurgitation, and all had normal LV dimension and systolic function. There was no recurrent arch obstruction. Single-stage surgical repair of CoA-AVSD is feasible and reasonable. Survival and freedom from reintervention in our cohort approximate those outcomes of two-stage repair with durable left AV valve function and no recurrent arch obstruction. These patients are frequently syndromic and demonstrate mortality risk from non-cardiac causes. Consideration of a single-staged approach is warranted for appropriate patients with CoA-AVSD.

Visualization and Quantification of the Unrepaired Complete Atrioventricular Canal Valve Using Open-Source Software

Repair of complete atrioventricular canal (CAVC) is often complicated by residual left atrioventricular valve regurgitation. The structure of the mitral and tricuspid valves in biventricular hearts has previously been shown to be associated with valve dysfunction. However, the three-dimensional (3D) structure of the entire unrepaired CAVC valve has not been quantified. Understanding the 3D structure of the CAVC may inform optimized repair. Novel open-source work flows were created in SlicerHeart for the modeling and quantification of CAVC valves on the basis of 3D echocardiographic images. These methods were applied to model the annulus, leaflets, and papillary muscle (PM) structure of 35 patients (29 with trisomy 21) with CAVC using transthoracic 3D echocardiography. The mean leaflet and annular shapes were calculated and visualized using shape analysis. Metrics of the complete native CAVC valve structure were compared with those of normal mitral valves using the Mann-Whitney U test. Associations between CAVC structure and atrioventricular valve regurgitation were analyzed. CAVC leaflet metrics varied throughout systole. Compared with normal mitral valves, the left CAVC PMs were more acutely angled in relation to the annular plane (P<.001). In addition, the anterolateral PM was laterally and inferiorly rotated in CAVC, while the posteromedial PM was more superiorly and laterally rotated, relative to normal mitral valves (P<.001). Lower native CAVC atrioventricular valve annular height and annular height-to-valve width ratio before repair were both associated with moderate or greater left atrioventricular valve regurgitation after repair (P<.01). It is feasible to model and quantify 3D CAVC structure using 3D echocardiographic images. The results demonstrate significant variation in CAVC structure across the cohort and differences in annular, leaflet, and PM structure compared with the mitral valve. These tools may be used in future studies to catalyze future research intended to identify structural associations of valve dysfunction and to optimize repair in this vulnerable and complex population.

Non-Invasive Surveillance for Cardiac Allograft Rejection Using an Echocardiographic Rejection Score in Pediatric Heart Transplant Recipients

<h3>Purpose</h3> Cardiac allograft rejection post heart transplant (HT) remains a significant cause of morbidity and mortality in pediatric HT recipients. Endomyocardial biopsy is the gold standard for diagnosis, but is invasive, expensive, and not without risks. We examine the correlation between a non-invasive echocardiographic ejection score (ERS) and clinical and echocardiographic (echo) evidence of rejection. <h3>Methods</h3> All pediatric HT patients followed at our institution who had surveillance echo using the ERS were retrospectively enrolled. Rejection is defined as any changes in clinical or echo parameters resulting in augmented immunosuppression or administration of steroids, biologics, or plasmapheresis. ERS, left ventricular relaxation index (LVRI), RR interval, diastolic time, and atrioventricular valve regurgitation were obtained from the echo reports. The ERS is scored from 0-10 and is stratified as follows: 0-4: no rejection; 5-6: possible rejection necessitating closer follow up or consideration of other clinical factors; ≥7: automatic rejection. <h3>Results</h3> Sixty-six patients were followed over an 8 year period. Mean age at HT was 6.81 years (range 22 days - 21 years). Thirty-five (53%) patients were male. Primary indication for HT was cardiomyopathy in 54.5% (n=36), congenital heart disease in 44% (n=29), and other in 1.5% (n=1). Fifty-eight episodes of acute or ongoing rejection were treated based on clinical evidence. The ERS correlated weakly with LVRI (r = -.191, p < .01), RR interval (r = .117, p <.01), diastolic time (r = .159, p <.01), and new onset mitral or tricuspid regurgitation (MR/TR) (r = .168, p <.01). The ERS did not correlate with rejection identified by changes in clinical status alone (r = .015, p = .428). <h3>Conclusion</h3> ERS appears to be a useful non-invasive diagnostic tool for early detection of rejection in heart transplant pediatric patients. A larger study is needed to determine the diagnostic utility of this non-invasive ERS.

Incidence, Risk Factors, and Outcomes of Atrial Arrhythmias in Adult Patients With Atrioventricular Septal Defect.

This study aimed to assess the incidence, associated factors, and outcomes of atrial arrhythmias in adults with atrioventricular septal defect (AVSD). Data regarding atrial arrhythmias in adults with AVSD are particularly scarce. Data were analyzed from a multicentric cohort of adult patients with AVSD. Lifetime cumulative incidences of atrial arrhythmias were studied. Multiple logistic regression models were used to identify risk factors. A total of 391 patients (61.6% women) were enrolled with a mean age of 36.3 ± 16.3 years and a mean follow-up of 17.3 ± 14.2 years after initial surgical repair. Overall, 98 patients (25.1%) developed at least 1 episode of atrial arrhythmia at a mean age of 39.2 ± 17.2 years. The mean ages of patients at first episode of intra-atrial re-entrant tachycardia (IART)/ focal atrial tachycardia (FAT) and atrial fibrillation were 33.7 ± 15.3 and 44.3 ± 16.5 years, respectively. The lifetime risks for developing atrial arrhythmia to ages 20, 40, and 60 years were 3.7%, 17.8%, and 55.3%, respectively. IART/FAT was the leading arrhythmia until the age of 45, then atrial fibrillation surpassed IART/FAT. Age (odds ratio [OR]: 1.4; 95% confidence interval [CI]: 1.2-1.6), number of cardiac surgeries (OR: 4.1; 95%CI: 2.5-6.9), left atrial dilatation (OR: 3.1; 95%CI: 1.4-6.8), right atrial dilatation (OR: 4.1; 95%CI: 1.7-10.3), and moderate or severe left atrioventricular valve regurgitation (OR: 3.7; 95%CI: 1.2-11.7) were independently associated with a higher risk of atrial arrhythmias, whereas the type of AVSD and the age at repair were not. The occurrence of atrial arrhythmias was associated with pacemaker implantation (41.8% vs. 8.5%; P< 0.001), heart failure (24.5% vs. 1.0%; P< 0.001), and cerebrovascular accidents (11.2% vs. 3.4%; P = 0.007). The lifetime risk of atrial arrhythmias in patients with AVSD is considerable with more than half of patients developing≥1 atrial arrhythmia by the age of 60 and is associated with a significant morbidity. The risk in partial/intermediate AVSD is as high as in complete AVSD and is not impacted by age at repair.

An atrioventricular septal defect with an Ebsteinoid right atrioventricular valve

Abstract Funding Acknowledgements Type of funding sources: None. The combination of an atrioventricular septal defect (AVSD) with an Ebsteinoid atrioventricular valve is incredibly rare with only a handful of cases being described in the literature (1, 2). In this poster we describe the diagnosis of this anatomy in a neonate born at our centre. The baby was antenatally diagnosed with a chromosome 8p23.1 microdeletion which is associated with congenital heart disease and developmental delay (3). Although fetal echo had revealed a high suspicion of congenital heart disease, maternal BMI had prevented a precise diagnosis. The baby was born in good condition at thirty-nine weeks gestation weighing 2.4kg. She had been admitted to the neonatal unit where she was self-ventilating in room air with saturations above 90%. The echocardiogram was performed at one hour of age. The initial echo revealed a normal atrial arrangement with the heart in the left chest and the apex pointing to the left. There was normal pulmonary and systemic venous drainage. There was a complete AVSD with sizeable atrial and ventricular components. The left atrioventricular valve was dysplastic with at least moderate regurgitation. The right atrioventricular valve was displaced caudally and rotated into the right ventricular outflow tract. The right atrioventricular valve was also regurgitant. The right ventricle above the displaced atrioventricular valve was atrialized with the true right ventricle limited to the outlet portion. The great arteries were normally related. The ventricular component of the AVSD opened into the sub-pulmonary area along with two smaller muscular ventricular septal defects. These findings raised many clinical concerns including the insufficiency of the right ventricle to support a biventricular repair. Similarly, the severe left atrioventricular valve regurgitation precluded single ventricle palliative surgical techniques. After discussion between the multidisciplinary team and a second opinion sought from another centre, the family was counselled that this was a complex and life limiting form of congenital heart disease. Although surgical options were discussed with the family, the family opted for comfort care and the baby passed away at three weeks of age. Image 1: A complete AVSD with an Ebsteinoid right atrioventricular valve displaced caudally (a) resulting in an atrialized right ventricle (b). In addition, the left atrioventricular valve which straddles the ventricular septum is severely dysplastic (c) and regurgitant (d). Abstract Figure. Image 1: An Ebsteinoid AVSD

Echocardiographic Evaluation of Postoperative Coaptation Geometry of Left AV Valve in Complete Atrioventricular Septal Defect.

We sought to determine the difference in geometric parameters in the left atrioventricular valve (LAVV) postoperative complete atrioventricular septal defect (CAVSD) compared to the normal heart, and the correlation between geometric and functional parameters for detecting the mechanism of LAVV regurgitation (LAVVR) in CAVSD. LAVV geometric parameters based on complete and acceptable quality echocardiograms of 18 patients with repaired CAVSD compared with 17 normal controls. LAVVR severity was also quantified by indexed vena contracta (I-VC) (mm) and % jet area/left atrium area (% Jet/LA), and the correlation with LAVV parameters in the CAVSD group was investigated. In the CAVSD group, the posterior closing angle (Pc) was nearly the same as the anterior closing angle (Ac), yet in the normal heart, the Pc angle was double the Ac angle. The anterior opening angle (Ao) and posterior-to-anterior leaflet diameter ratio (a/p) in the CAVSD group was also significantly smaller. The CAVSD group also had a shorter indexed coaptation length (I-CL) and indexed tenting height (I-TH). Displacement length (ΔD) differed completely between the CAVSD and Normal groups, and also showed a strong positive correlation to the functional parameters of LAVVR (% Jet/LA: r = .70, P = .02; I-VC: r = .60, P = .02). The parameters in this study were applicable to CAVSD AV valve coaptation characteristics. We introduced 2 novel measures that may provide important insights into the differences in geometry and performance of the LAVV in repaired CAVSD as compared to normal hearts.