After Atrioventricular Septal Defect Research Articles

The role of abnormal subaortic morphometry as a substrate for left ventricular outflow tract obstruction following atrioventricular septal defect repair.

Although left ventricular outflow tract (LVOT) obstruction is a recognized risk after atrioventricular (AV) septal defect (AVSD) repair, quantitative assessments to define the substrate of the obstruction are lacking. Morphometric analyses were based on measurements from early 2-dimensional echocardiographic scans (within 3 months postoperatively) for 117 patients (82 CAVVO = common AV valve; 35 SAVVO = separate AV valve orifices), which were compared to 50 age/weight matched controls (atrial septal defect/ventricular septal defect). Late echocardiographic analyses were performed in 57 patients with AVSD (follow-up range, 1.2-10.7 years). Adequate z scores (above -2.5) were observed in 109 (93%) patients with AVSD at the aortic annulus and in 89 (76%) with AVSD in the subaortic area. Compared to the control group, patients with AVSD had lower median z scores at the aortic annulus (-0.64 vs 0.60; P < 0.001) and the subaortic areas (-1.48 vs 0.59; P < 0.001), disproportionate subaortic/aortic annulus ratio <1.00 (67% vs 22%; P < 0.001), narrower annuloaortic-septal angle (94.0 vs 104.0; P < 0.001) and annuloaortic left AV valve angle (78.0 vs 90.0; P < 0.001). Compared to patients with CAVVO, those with SAVVO had narrower annuloaortic-septal angles (P = 0.022) that persisted at late analysis, with lower subaortic/aortic annular ratios (P = 0.039). In patients with CAVVO, lower early postoperative subaortic z scores were found following modified single-patch repairs (median -2.12 vs -1.02 in two-patch repairs; P = 0.004). A total of 6/117 (5%) patients (4 CAVVO, 5% and 2 SAVVO, 6%) required reoperations for LVOT obstruction (mean 6.9 years postoperatively), with no difference in morphology or types of operations. Despite having adequate z scores, patients with AVSD demonstrated abnormal LVOT morphometrics early postoperatively. Besides intrinsic morphology, repair techniques may have an impact on postoperative LVOT morphometrics and requires further evaluation.

Fate of significant left atrioventricular valve residual gradient after atrioventricular canal repair

Reoperation for left atrioventricular valve (LAVV) dysfunction after complete repair is a well-known situation. Although regurgitation is the main cause, few studies review the fate of postoperative residual stenosis. Clinical and echographic outcomes of patients with significant early postoperative gradient of left atrioventricular valve after atrioventricular septal defect (AVSD) repair. Between January 2000 and December 2019, 887 with an AVSD repaired were reviewed. Forty-one of them presented a LAVV mean gradient of more or equal than 5 mmHg at the first postoperative echo. The follow-up postoperative data were obtained from hospital records and cardiologists correspondences. We studied the postoperative mean gradient of the LAVV at discharge, one and tree months, one and five years after the repair. The median follow-up was 51 months (3–123 months). Among the 41 patients included 15 had a complete AVSD (cAVSD) and 26 had a partial AVSD p(AVSD). The median age and weight at repair were 4.5 (2.4–107.5) months, 4.8 (4–19) kg for the complete AVSD (cAVSD) and 50.8 (4.2–200.4) months, 15 (4.7–38.6) kg for the partial AVSD (pAVSD), respectively. Six (40) cAVSD were diagnosed with Down syndrome and 5 (33.3) had a pulmonary artery banding before the repair. There was no reoperation for LAVV stenosis neither for regurgitation. Patients remained asymptomatic with normal range of pulmonary pressure. One patient was reoperated for left ventricular outflow tract obstruction. The median (range) LAVV gradient measured were the following at first postoperative echo, discharge, one and 3 months, one an, last follow-up: 5 (3–10), 4.5 (2–5), 3.4 (1.5–6) (Table 1, Fig. 1). The difference between the first postoperative echo gradient and the last one was not significant (P = 0.47). Significant LAVV gradient after AVSD is not uncommon. Overall trend is the stability of the gradient with normal pulmonary pressure or even decreasing. Isolated LAVV mean gradient between 5 to 10 mmHg does not seem to be an indication for reclamping. A moderate gradient due to the zone of apposition closure and commissural annuloplasty is acceptable when needed to obtain a competent LAVV.

Abstract 15099: Limitations of Predicting Postoperative Left Atrioventricular Valve Regurgitation by Transesophageal Echocardiography

Introduction: After atrioventricular septal defect (AVSD) repair, the degree of post-repair left atrioventricular valve regurgitation (LAVVR) at discharge is associated with need for re-intervention. Knowledge of the relationship between post-repair transesophageal echocardiogram (TEE) and discharge transthoracic echocardiogram (TTE) LAVVR could influence intraoperative decision-making. At present, this relationship has not been well-defined and to assess it, we undertook a retrospective study of a large cohort of AVSD patients. Methods: Between 11/2012-12/2018, 96 AVSD patients underwent surgical repair. Data included patient demographics, clinical characteristics, preoperative LAVV anomalies, surgical procedure and outcomes. LAVVR was graded as less than moderate or moderate and greater with statistical blinding techniques employed. Cohen’s kappa statistics were calculated to measure interobserver variability. Association between parameters was tested using univariate analysis. Results: Median age and weight at surgery for complete AVSD patients (68%) was 5 months and 5.6 kg respectively; for partial AVSD (32%), median age and weight was 27 months and 11.9 kg respectively. The median interval between echo studies was 6 days (IQR 4-12 days) with median length of stay of 8 days (IQR 5-16 days). There were no deaths. Post-repair TEE revealed less than moderate LAVVR in 86 patients (90%) and moderate or greater in 10 (10%). Overall, there was a discrepancy in LAVVR seen at discharge in 25 patients (26%) with 23/25 (92%) demonstrating a higher grade of LAVVR. Two patients underwent reoperation before discharge due to severe LAVVR. In univariate analysis, significant predictors for LAVVR discrepancy were younger age (p=0.032) and lower weight (p=0.032) at repair; preoperative LAVVR grade was a significant predictor for worsening LAVVR (p=0.038). Conclusions: Discrepancy between LAVVR grade on post-repair TEE and discharge TTE was present in 26% of AVSD patients with the vast majority changing to a higher grade. This important finding with implications for intraoperative decision-making should prompt further investigations into preoperative LAVV anomalies, surgical technique and postoperative factors influencing valvar function.

An Analysis of Hospital Mortality After Cardiac Operations in Children With Down Syndrome

Children with Down syndrome (DS) have lower mortality compared to nonsyndromic (NS) children after atrioventricular septal defect (AVSD) repair. Limited data exist regarding hospital mortality and utilization after other congenital heart disease (CHD) operations in DS. We compared hospital mortality and utilization after CHD operations in both populations and hypothesized that the survival benefit in children with DS is not consistent across CHD lesions. The Texas Inpatient Public Use Datafile was queried for all patients <18 years old undergoing operations for CHD between 1999 and 2016. Hospital mortality, length-of-stay and charges were compared between DS and NS groups, stratified by CHD operation using mixed-effects multivariable analyses and propensity score matching analyses adjusting for prematurity, low birth weight, age, and sex. Over the 18-year period, 2841 cases with DS underwent CHD operations compared to 25,063 NS cases. The most common types of interventions performed in DS were AVSD repair, isolated ventricular septal defect (VSD) repair and tetralogy of Fallot (TOF) repair. By multivariable analyses, DS was associated with lower mortality after isolated AVSD repair (RR 0.40 [IQR 0.20-0.79]), and higher hospital mortality after bidirectional Glenn anastomosis (BDG) (RR 5.17 [IQR 2.10-12.77]) and TOF/pulmonary atresia repair (RR 9.71 [IQR 2.16-43.68]) compared to NS children. Similar results were noted using propensity score matching. Children with DS had lower mortality after AVSD repair than NS children, but higher mortality after operations for BDG and TOF/pulmonary atresia. Further study is needed to determine if the presence of pulmonary hypertension in DS modifies the association between DS and mortality depending on cardiac lesion.

Incidence and risk factors of post-operative arrhythmias and sudden cardiac death after atrioventricular septal defect (AVSD) correction: Up to 47years of follow-up.

Atrioventricular septal defect (AVSD) has an incidence of 4-5.3 per 10.000 live births and is associated with Down syndrome (DS). Data on arrhythmias and sudden cardiac death (SCD) after AVSD correction is scarce. To analyse the incidence of post-operative arrhythmias and SCD after AVSD correction and explore risk factors. This is a retrospective multicenter study including patients after biventricular AVSD correction. Univariate and multivariate analyses were performed to explore risk factors. A total of 415 patients were included with a mean follow-up duration of 9years (range; <30days-47years). Early post-operative SVTs were documented in 33 patients (8%) and late post-operative SVTs in 15 patients (3.6%). Non-syndromic AVSD (p=0.022, HR=2.64; 95% CI=1.15-6.04) and cAVSD (p=0.005, HR=3.7; 95% CI=1.39-7.51) were independent risk factors for early post-operative SVTs and significant more late post-operative SVTs occurred in non-syndromic patients (p=0.016, HR=6.38; 95% CI=1.42-28.71) and in pAVSD (p=0.045, HR=3.703; 95% CI=1.03-13.32). Fifteen patients (3.6%) received a pacemaker. Non-syndromic AVSD (p=0.008, HR=15.82; 95% CI=2.04-122.47), pAVSD (p=0.017, HR=6.26; 95% CI=1.39-28.28) and re-operation (p=0.007, HR=4.911; 95% CI=1.54-15.64) were independent risk factors for postoperative pacemaker implantation. Late life-threatening ventricular arrhythmias and SCD occurred in 0.5% and 1.7% respectively. There is good long-term survival after AVSD correction and incidence of SCD is low. Non-syndromic AVSD and cAVSD are independent risk factors for early post-operative SVTs. Non-syndromic AVSD patients have significant more early 3rd degree AVB and late post-operative SVTs. Non-syndromic patients with partial AVSD who have undergone reoperation have a significant higher risk of pacemaker implantation.

Elevated energy loss in diastolic left ventricular inflow corresponds to an increase in kinetic energy in patients with a repaired atrioventricular septal defect: Quantification from 4D Flow MRI

Background Patients after atrioventricular septal defect (AVSD) repair may present abnormalities in valve morphology and subsequently develop altered left ventricular (LV) inflow patterns [1]. This may lead to energy loss and affect the kinetic energy in the LV. We aimed to quantify diastolic viscous energy loss and kinetic energy in the LV and to evaluate their association in AVSDrepaired patients compared to healthy controls using 4D Flow MRI.

Disturbed diastolic left ventricular inflow vortex ring formation in patients with corrected atrioventricular septal defect: quantitative three-dimensional vortex core analysis from 4DFlow MRI

Background Vortex formation in the left ventricle (LV) is suggested to contribute to efficient blood pumping and altered vortex formation is associated with diastolic dysfunction. Patients after atrioventricular septal defect (AVSD) correction present abnormalities in valve morphology and subsequently develop altered inflow patterns (Calkoen et al. JMRI 2014), which may disturb normal vortex formation. We aimed to analyze vortex ring formation in AVSD-corrected patients compared to healthy controls and to evaluate association with inflow angle.

Impact of disturbed diastolic vortex formation on viscous energy loss in the left ventricle: Quantitative 4D Flow MRI analysis of healthy controls and repaired atrioventricular septal defect patients

Background Vortex formation in the left ventricle (LV) is suggested to contribute to efficient blood pumping and minimization of energy loss. Patients after atrioventricular septal defect (AVSD) repair may present abnormalities in valve morphology and subsequently develop altered LV inflow patterns [1], in which normal vortex formation may be disturbed. This may lead to energy loss. We aimed to analyze the association between disturbed vortex ring formation during diastole and viscous energy loss during diastole in AVSD-corrected patients compared to healthy controls.

Characterization and improved quantification of left ventricular inflow using streamline visualization with 4DFlow MRI in healthy controls and patients after atrioventricular septal defect correction.

To evaluate trans-left atrioventricular valve (LAVV) blood flow and optimize left ventricular inflow quantification in healthy controls and patients after atrioventricular septal defect (AVSD) correction. Twenty-five patients after AVSD correction and 25 controls underwent 4DFlow MRI. Using streamline visualization in four- and two-chamber views, inflow direction at early and late filling was defined at the annulus level and at the peak inflow velocity (PIV) level. Trans-LAVV flow volume and velocity were assessed from a static 2D-multiplanar-reformat (MPR), a 4D-MPR tracking LAVV annulus and a 4D-MPR tracking the PIV-level, angulated perpendicular to the inflow. In patients, on average 9° more laterally directed inflow was found at the PIV-level compared to controls. In controls, 4DFlow velocity mapping with LAVV annulus tracking resulted in lower absolute error with aortic flow (3 (1-8) mL) than with static 2D-MPR (7 (4-16) mL, P = 0.001). In patients, 4D-MPR tracking the PIV-level, resulted in lower absolute error with aortic flow (2 (1-4) mL) than with 4D-MPR LAVV annulus tracking (6 (2-10) mL, P = 0.003). Streamline visualization of 4DFlow MRI data revealed dynamic trans-LAVV inflow and more lateral flow after AVSD correction. Streamline visualization improved trans-LAVV flow quantification as the positioning and angulation of the measurement plane was optimized, allowing an accurate assessment of left ventricular inflow.

Characterization and quantification of dynamic left atrioventricular valve regurgitation after atrioventricular septal defect correction with 4D Flow MRI and retrospective valve tracking

Background Regurgitation of the left atrio-ventricular valve (LAVV) is common after atrioventricular septal defect (AVSD) correction and up to 15% of the patients require surgery of the LAVV during follow-up. Reliable quantification of LAVV regurgitation after AVSD correction is essential for indicating surgical correction, but has shown to be difficult using echocardiography. 4DFlow MRI with retrospective valve tracking allows visualization and quantification of trans-valvular blood flow. The aim of cuurent study was to describe dynamic behaviour of regurgitant jets of the LAVV after AVSD correction and to quantify severity of regurgitation using 4DFlow MRI with retrospective valve tracking. Methods

Reoperation for Left Atrioventricular Valve Regurgitation After Atrioventricular Septal Defect Repair

Left atrioventricular valve regurgitation (LAVVR) is a major cause of morbidity after atrioventricular septal defect (AVSD) repair. This study evaluates the outcomes of repair and replacement of the left atrioventricular valve after AVSD correction, as well as factors predictive of durability of valve repair. Between January 1983 and March 2007, 31 patients underwent reoperation for LAVVR after AVSD repair (23 valve repairs and 8 valve replacements). Median age at primary repair was 5.0 months and time to reoperation was 5.0 months. The distribution of AVSD morphology was 9 primum, 5 transitional, and 17 complete. Early postoperative mortality was 6.4% (2 of 31). Survival at 10 years was 88.1%. At a mean follow-up of 8.2 years, 86% of hospital survivors were in New York Heart Association class I. Overall freedom from reintervention at 10 years was 67.2%. Among patients undergoing primary repair, 6 of 23 underwent subsequent replacement. Follow-up LAVVR in those who did not require subsequent valve replacement was mild or less in 92.8%. Factors that demonstrated a trend toward durable repair included the use of patch augmentation rather than primary cleft closure (p = 0.02) and earlier timing to repair (less than 2 months; p = 0.03). Significant cardiomyopathy developed in 21.4% of patients after prosthetic valve replacement (3 of 14). Surgical management of LAVVR after AVSD repair can be performed with excellent midterm outcomes. However, both repair and replacement are associated with a high incidence of reoperation. Nonetheless, an aggressive reparative approach should be pursued to avoid the morbidity of pediatric left atrioventricular valve replacement that includes anticoagulation, inevitable reoperation, and cardiomyopathy.

Real-time transthoracic three-dimensional echocardiography provides additional information of left-sided AV valve morphology after AVSD repair

Aims The purpose of this study was to assess the feasibility of real-time 3D echocardiography (RT-3DE) data acquisition in adult patients after atrioventricular septal defect (AVSD) repair and to evaluate whether RT-3DE has additional value over 2D echocardiography, regarding morphology and function of the left-sided AV valve (LAVV). Methods Twenty consecutive patients with surgically corrected partial or complete AVSD were enrolled in this study. The 3DE data sets were acquired with the Hewlett-Packard Sonos 7500 echo system (Philips Medical Systems, Andover, MA, USA). Images were reviewed off-line with assistance of TomTec Echoview 5.2 software (TomTec Inc., Munich, Germany) by experienced observers. En face reconstructions, from, respectively, the ventricular and atrial view, were made to evaluate the LAVV morphology and motion. Results 3DE reconstruction of the LAVV was feasible in 17 of 20 patients (85%). Mean time of 3DE acquisition was 9 ± 6 min. The quality of the 3DE images was optimal in 35%, good in 30%, sufficient in 20% and insufficient in 15%. Identification of the LAVV structures was importantly better facilitated from a ventricular view. Accurate identification of LAVV morphology was possible in all 17 patients (85%). Relationship of the LAVV and the abnormal position of the LVOT was easier to evaluate from the 3DE reconstructions than from 2D echo. Conclusion This study demonstrate that RT-3DE is feasible in daily practice and provides new insight into the dynamic morphology of the left-sided AV valve and LVOT anatomy after AVSD repair.

Three-dimensional echocardiographic analysis of valve anatomy as a determinant of mitral regurgitation after surgery for atrioventricular septal defects

Mitral regurgitation (MR) is a significant complication after atrioventricular septal defect (AVSD) surgery. The relation of the valve leaflet morphology and the MR mechanism remains a conundrum. Two-dimensional echocardiography depicts leaflet edges, whereas volume-rendered 3-dimensional echocardiography provides direct visualization of the surface areas of the mitral valve leaflets. This study examines the relation of mitral valve anatomy as determined by 3-dimensional echocardiography with MR origins in patients after AVSD repair. Twenty-seven patients with AVSD surgery and Doppler color MR were prospectively enrolled (median age was 5 years and 16 patients had Down syndrome). Doppler color flow imaging of the MR jet and 3-dimensional echocardiography of the mitral valve were performed with a probe in the transthoracic or transesophageal position. Enface 3-dimensional views of the mitral valve from the left atrium were reconstructed. Analysis of the 3-dimensional data was possible in 21 of the 27 patients. Mean area ratios of the 3 mitral leaflets were calculated (superior 40 ± 7%, inferior 35 ± 5%, mural 25 ± 6%). Both intra and interobserver variability on the area measurements were <5%. In 12 patients (group 1) the jet appeared to emanate medially from the region of coaptation of the superior and inferior components of the anterior leaflet. In 9 patients (group 2) the jet emanated more laterally from the region toward the mural leaflet. The area ratios of the inferior leaflet were 32 ± 4% in group 1 and 38 ± 6% in group 2 (p = 0.02). The area ratios of the mural leaflet were 28 ± 5% in group 1 and 21 ± 5% in group 2 (p = 0.007). The superior leaflet area ratio was not different in groups 1 and 2, 40 ± 9% and 41 ± 6%, respectively. Three-dimensional echocardiography provides new insight into the anatomic determinants of MR following AVSD surgery.