Common Atrium or Atrioventricular Septal Defect: What Is in a Name?
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ABSTRACT:
SUBMITTER: Agarwal D
PROVIDER: S-EPMC8713003 | biostudies-literature | 2021 Dec
REPOSITORIES: biostudies-literature
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Common Atrium or Atrioventricular Septal Defect: What Is in a Name?
Agarwal Deepmala D Lakhdhir Sarifa S Reveler Elaine E O'Sullivan Lynne L
CASE (Philadelphia, Pa.) 20211016 6
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Project description:BackgroundA partial atrioventricular septal defect (AVSD) with a hypoplastic left ventricle and common atrium is a rare combination of cardiac anomalies that can be associated with Ellis-van Creveld (EVC) syndrome.Case summaryA female neonate with EVC syndrome was diagnosed with an unbalanced AVSD and hypoplastic left ventricle. Pulmonary artery banding and ductus ligation were performed at 23 days after birth. The postoperative course was complicated by moderate atrioventricular valve (AVV) regurgitation and low cardiac output. On postoperative Day 6, emergent extracorporeal membrane oxygenation (ECMO) was performed to treat acute circulatory failure. Under suspicion of systemic ventricular outflow tract obstruction, the Damus-Kaye-Stansel procedure with a systemic-to-pulmonary artery shunt and AVV plasty were performed. Intraoperatively, no ventricular septal defect was found. The right and left AVV orifices were found to be separated. Postoperatively, the patient could not be weaned from cardiopulmonary bypass and continued to receive ECMO support. Eight days postoperatively, a right ventricle-to-pulmonary artery shunt and division of the systemic-to-pulmonary artery shunt were performed to increase the pulmonary blood flow. On postoperative Day 5, the ECMO was successfully removed under continuous infusion of adrenalin, but the patient died of severe renal failure 4 days later. The parents consented to autopsy. The heart was permanently preserved by perfusion-distention fixation and wax infiltration.DiscussionWe reported a rare combination of cardiac defects of common atrium, partial AVSD, and hypoplastic left ventricle associated with EVC syndrome. Accurately diagnosing the presence of ventricular septal defect is essential part in determining surgical treatment strategy.
| S-EPMC11770385 | biostudies-literature
Project description: Not available
| S-EPMC1861295 | biostudies-literature
Project description:BackgroundSurgical and perioperative improvements permit earlier repair of partial and transitional atrioventricular septal defects (AVSD). We sought to describe contemporary outcomes in a multicenter cohort.MethodsWe studied 87 patients undergoing primary biventricular repair of partial or transitional AVSD between June 2004 and February 2006 across seven North American centers. One-month and 6-month postoperative data included weight-for-age z-scores, left atrioventricular valve regurgitation (LAVVR) grade, residual shunts, and left ventricular ejection fraction. Paired methods were used to assess 6-month change.ResultsMedian age at surgery was 1.8 years; median weight z-score was -0.88. Median days for ventilation were 1, intensive care 2, and hospitalization 5, all independent of age, with 1 in-hospital death. At 1 month, 27% (16 of 73) had ejection fraction less than 55%; 20% (17 of 87) had significant LAVVR; 2 had residual shunts; 1 each had subaortic stenosis and LAVV stenosis. At 6 months (n = 60), there were no interim deaths, reinterventions, or new development of subaortic or LAVV stenosis. Weight z-score improved by a median 0.4 units (p < 0.001), especially for underweight children less than 18 months old. Left atrioventricular valve regurgitation occurred in 31% (change from baseline, p = 0.13), occurring more frequently in patients repaired at 4 to 7 years (p = 0.01). Three patients had ejection fraction less than 55%, and 1 had a residual atrial shunt.ConclusionsSurgical repair for partial/transitional AVSD is associated with low morbidity and mortality, short hospital stays, and catch-up growth, particularly in underweight children repaired between 3 and 18 months of age. Left atrioventricular valve regurgitation remains the most common residual defect, occurring more frequently in children repaired after 4 years of age.
| S-EPMC2900854 | biostudies-literature
Project description: Not available
| S-EPMC5070315 | biostudies-literature
Project description:ObjectiveAlthough a narrow left ventricular outflow tract in atrioventricular septal defect is related to its intrinsic morphology, the contribution from the repair technique remains to be quantified.MethodsA total of 108 patients with an atrioventricular septal defect with a common atrioventricular valve orifice were divided into 2 groups: 2-patch (N = 67) and modified 1-patch (N = 41) repair. The left ventricular outflow tract morphometric was analyzed by quantifying the degree of disproportion between subaortic and aortic annular dimensions (disproportionate morphometrics ratio was defined as ≤ 0.9). Z-scores (median, interquartile range) were further analyzed in a subset of 80 patients with immediate preoperative and postoperative echocardiography. A total of 44 subjects with ventricular septal defects served as controls.ResultsBefore repair, 13 patients (12%) with an atrioventricular septal defect had disproportionate morphometrics (vs 6 [14%] ventricular septal defect P = .79), but the subaortic Z-score (-0.53, -1.07 to 0.06) was lower than the ventricular septal defect (0.07, -0.57 to 1.17; P < .001). After repair, both 2-patch (8 [12%] preoperatively vs 25 [37%] postoperatively; P = .001) and modified 1-patch (5 [12%] vs 21 [51%], P < .001) procedures showed a greater degree of disproportionate morphometrics. Both 2-patch (postoperatively -0.73, -1.56 to 0.08 vs preoperatively -0.43, -0.98 to 0.28; P = .011) and modified 1-patch (-1.42, -2.63 to -0.78 vs -0.70, -1.18 to -0.25; P = .001) procedures also demonstrated lower subaortic Z-scores postrepair. The postrepair subaortic Z-scores were lower in the modified 1-patch group (-1.42 [-2.63 to -0.78]) compared with the 2-patch group (-0.73 [-1.56 to 0.08]; P = .004). Low postrepair subaortic Z-scores (<-2) were observed in 12 patients (41%) in the modified 1-patch group and 6 patients (12%) in the 2-patch group (P = .004).ConclusionsSurgical correction resulted in greater disproportionate morphometrics seen immediately postrepair. The impact on the left ventricular outflow tract was observed in all repair techniques, with a greater burden seen after modified 1-patch repair.Video abstractThis morphometric study in AVSD with common atrio-ventricular valve orifice confirmed further derangements of LV outflow tract morphometrics immediately after surgical repair.
| S-EPMC10328763 | biostudies-literature
Project description:AimsThe incidence of atrial tachyarrhythmias is high in patients with atrioventricular septal defect (AVSD). No specific data on catheter ablation have been reported so far in this population. We aimed to describe the main mechanisms of atrial tachyarrhythmias in patients with AVSD and to analyse outcomes after catheter ablation.Methods and resultsThis observational multi-centric cohort study enrolled all patients with AVSD referred for catheter ablation of an atrial tachyarrhythmia at six tertiary centres from 2004 to 2022. The mechanisms of the different tachyarrhythmias targeted were described and outcomes were analysed. Overall, 56 patients (38.1 ± 17.4 years, 55.4% females) were included. A total of 87 atrial tachyarrhythmias were targeted (mean number of 1.6 per patient). Regarding main circuits involved, a cavo-annular isthmus-dependent intra-atrial re-entrant tachycardia (IART) was observed in 41 (73.2%) patients and an IART involving the right lateral atriotomy in 10 (17.9%) patients. Other tachyarrhythmias with heterogeneous circuits were observed in 13 (23.2%) patients including 11 left-sided and 4 right-sided tachyarrhythmias. Overall, an acute success was achieved in 54 (96.4%) patients, and no complication was reported. During a mean follow-up of 2.8 ± 3.8 years, 22 (39.3%) patients had at least one recurrence. Freedom from atrial tachyarrhythmia recurrences was 77.5% at 1 year. Among 15 (26.8%) patients who underwent repeated ablation procedures, heterogeneous circuits including bi-atrial and left-sided tachyarrhythmias were more frequent.ConclusionIn patients with AVSD, most circuits involve the cavo-annular isthmus, but complex mechanisms are frequently encountered in patients with repeated procedures. The acute success rate is excellent, although recurrences remain common during follow-up.
| S-EPMC10516589 | biostudies-literature
Project description: Not available
| S-EPMC9794495 | biostudies-literature
Project description:BackgroundThere is no relevant study on landmarks detection, one of the Convolutional Neural Network algorithms, in the field of fetal echocardiography (FE). This study aimed to explore whether automatic landmarks detection could be used in FE correctly and whether the atrial length (AL) to ventricular length (VL) ratio (AVLR) could be used to diagnose atrioventricular septal defect (AVSD) prenatally.MethodsThis was an observational study. Two hundred and seventy-eight four-chamber views in end diastole, divided into the normal, AVSD, and differential diagnosis groups, were retrospectively included in this study. Seven landmarks were labeled sequentially by the experts on these images, and all images were divided into the training and test sets for normal, AVSD, and differential diagnosis groups. U-net, MA-net, and Link-net were used as landmark prediction neural networks. The accuracy of the landmark detection, AL, and VL measurements, as well as the prenatal diagnostic effectiveness of AVLR for AVSD, was compared with the expert labeled.ResultsU-net, MA-net, and Link-net could detect the landmarks precisely (within the localization error of 0.09 and 0.13 on X and Y axis) and measure AL and VL accurately (the measured pixel distance error of AL and VL were 0.12 and 0.01 separately). AVLR in AVSD was greater than in other groups (P<0.0001), but the statistical difference was not obvious in the complete, partial, and transitional subgroups (P>0.05). The diagnostic effectiveness of AVLR calculated by three models, area under receiver operating characteristic curve could reach 0.992 (0.968-1.000), was consistent with the expert labeled.ConclusionsU-net, Link-net, and MA-net could detect landmarks and make the measurements accurately. AVLR calculated by three neural networks could be used to make the prenatal diagnosis of AVSD.
| S-EPMC10839271 | biostudies-literature
Project description:BackgroundLeft ventricular outflow tract stenosis and atrioventricular valve regurgitation are often problems encountered in adulthood after complete atrioventricular septal defect repair. The surgical approach and indications for managing long-term outcomes such as left atrioventricular valve regurgitation and left ventricular outflow tract stenosis after complete atrioventricular septal defect repair have been discussed.Case presentationA 23-year-old woman with intellectual disability was diagnosed with complete atrioventricular septal defect and underwent two-patch repair without cleft closure in childhood. Follow-up examination in adulthood demonstrated moderate left-sided atrioventricular valve regurgitation and left ventricular outflow tract stenosis with a circumferential ridge (peak velocity, 3.7 m/s; pressure gradient, 54 mmHg). Intraoperative findings showed a circumferential ridge under the aortic valve, and we removed the ridge. In addition, a cleft was present at the anterior leaflet, and we completely closed the cleft. Anticoagulation therapy was not initiated, and no embolic complications occurred. Follow-up echocardiography demonstrated no ridge under the aortic valve and only mild-range left AVVR.ConclusionsWe successfully performed surgical treatment without valve replacement or anticoagulation therapy in a patient with poor medical compliance. Delayed reoperation leads to degeneration of the valve structure and makes more difficult to repair. Atrioventricular valve regurgitation should be evaluated in combination with based on the etiology of the regurgitation especially cleft related or not, in addition to the dilatation annulus, cleft size, and depth of the leaflet coaptation depth, and associated other valve diseases.
| S-EPMC11443635 | biostudies-literature
Project description:BackgroundLeft atrioventricular valve (LAVV) stenosis following an atrioventricular septal defect (AVSD) repair is a rare but potentially life-threatening complication. While echocardiographic quantification of diastolic transvalvular pressure gradients is paramount in the evaluation of a newly corrected valve function, it is hypothesized that these measured gradients are overestimated immediately following a cardiopulmonary bypass (CPB) due to the altered hemodynamics when compared to postoperative valve assessments using awake transthoracic echocardiography (TTE) upon recovery after surgery.MethodsOut of the 72 patients screened for inclusion at a tertiary center, 39 patients undergoing an AVSD repair with both intraoperative transesophageal echocardiograms (TEE, performed immediately after a CPB) and an awake TTE (performed prior to hospital discharge) were retrospectively selected. The mean (MPGs) and peak pressure gradients (PPGs) were quantified using a Doppler echocardiography and other measures of interest were recorded (e.g., a non-invasive surrogate of the cardiac output and index (CI), left ventricular ejection fraction, blood pressures and airway pressures). The variables were analyzed using the paired Student's t-tests and Spearman's correlation coefficients.ResultsThe MPGs were significantly higher in the intraoperative measurements when compared to the awake TTE (3.0 ± 1.2 vs. 2.3 ± 1.1 mmHg; p < 0.01); however, the PPGs did not significantly differ (6.6 ± 2.7 vs. 5.7 ± 2.8 mmHg; p = 0.06). Although the assessed intraoperative heart rates (HRs) were also higher (132 ± 17 vs. 114 ± 21 bpm; p < 0.001), there was no correlation found between the MPG and the HR, or any other parameter of interest, at either time-point. In a further analysis, a moderate to strong correlation was observed in the linear relationship between the CI and the MPG (r = 0.60; p < 0.001). During the in-hospital follow-up period, no patients died or required an intervention due to LAVV stenosis.ConclusionsThe Doppler-based quantification of diastolic transvalvular LAVV mean pressure gradients using intraoperative transesophageal echocardiography seems to be prone to overestimation due to altered hemodynamics immediately after an AVSD repair. Thus, the current hemodynamic state should be taken into consideration during the intraoperative interpretation of these gradients.
| S-EPMC10001237 | biostudies-literature