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Inferior sinus venosus atrial septal defect (SVASD) is the rarest form of the atrial septal defect (ASD) and can sometimes go unnoticed. Although this defect can be associated with other congenital anomalies, its association with hypoplasia of the posterior mitral leaflet is extremely rare. In this case, we present a woman with a history of surgery for an ostium secundum ASD who exhibited persistent right heart chamber dilation. Echocardiography revealed hypoplasia of the posterior mitral leaflet, and cardiac magnetic resonance (CMR) imaging confirmed the presence of a previously undetected inferior sinus venosus ASD.

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This 10-minute video aims at improving skills for the structural assessment of the interatrial septum using 2-dimensional transthoracic echocardiography (TTE) to increase the ability to diagnose-or rule out-the different types of interatrial communications. Of the five types of lesions, this video focuses on ostium secundum atrial septal defect. This is the first video in our MicroLearning Video Series, designed to help a target audience of sonographers, general cardiologists, general practitioners who want to gain knowledge on fundamental cardiology, and technicians. View the video at https://vimeo.com/989145537/4898c3c590.

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BACKGROUND: Cleft in the mitral valve leaflet is a primary cause of congenital mitral regurgitation, stemming from developmental anomalies in the mitral valve and frequently associated with other congenital heart defects. Concurrent presence of cleft in mitral valve leaflet with atrial septal defect and ventricular septal defect is relatively rare. Echocardiography, especially transesophageal echocardiography, is essential in diagnosing cleft mitral valve leaflet and related congenital heart defects, providing critical, detailed imagery for accurate assessment. This study presents a young female patient whose anterior mitral cleft, along with atrial septal defect and ventricular septal defect, was revealed through three-dimensional transesophageal echocardiography. CASE PRESENTATION: A 25-year-old Iranian female, experiencing progressive dyspnea and diminished physical capacity over 3 months, was referred to our hospital. Initial examination and transthoracic echocardiography indicated severe mitral regurgitation. Further evaluation with transesophageal echocardiography corroborated these findings and identified a cleft in the anterior mitral valve leaflet, coupled with mild left ventricular enlargement and significant left atrial enlargement. The complexity of the patient's condition was heightened by the diagnosis of cleft mitral valve leaflet in conjunction with atrial septal defect and ventricular septal defect, showing the complex nature of congenital defects. CONCLUSION: This case emphasizes the critical role of transthoracic echocardiography in diagnosing cleft of mitral valve leaflet and associated cardiac anomalies, showcasing its superiority over transthoracic echocardiography for detailed visualization of cardiac structures. The identification of multiple congenital defects highlights the necessity for a comprehensive diagnostic approach to manage and treat patients with complex congenital heart diseases effectively. Future research should aim to refine diagnostic methodologies to enhance patient outcomes for cleft of mitral valve leaflets and related congenital conditions.

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Atrial Septal Defect closure in childhood and early adulthood has a good prognosis, but in older individuals the risk-benefit ratio is not as straightforward. We report a 57-year-old man who was easily fatigued when exercising. The cardiac examination revealed a wide and fixed splitting of S2, a pulmonary ejection systolic murmur grade III/VI, and increased jugular venous pressure. The transesophageal echocardiography showed Atrial Septal Defect secundum with a diameter of 20 mm, L-to-R shunt, and 5 mm, a thin and floppy inferior rim. The patient underwent surgical Atrial Septal Defect closure. The deficient posteroinferior rim occurs only in 3.3% of patients with secundum Atrial Septal Defect. This condition will enhance the likelihood of occluder dislodgement in the transcatheter closure approach. We learn from this case that surgical Atrial Septal Defect closure may be an option for elderly patients if there is an inadequate, thin, and floppy inferior rim or no comorbidities.

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Iatrogenic inferior vena cava (IVC)-left atrium (LA) shunt is a rare complication in atrial septal defect (ASD) surgery, caused by mistaking the Eustachian valve for the lower margin of the ASD. In this report, we describe the case of a 45-year-old woman who experienced circulatory collapse at termination of cardiopulmonary bypass during surgical IVC-LA shunt repair. Transesophageal echocardiography helped identify stenosis between the IVC and the right atrium, caused by a residual original incorrectly placed ASD patch. Removal of most of the patch led to improvement in circulatory failure.

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BACKGROUND: Atrial septal defects (ASD) are the most common type of adult congenital heart disease (ACHD) associated with a high risk developing of pulmonary arterial hypertension (PAH). ASD closure is not recommended in patients with PAH and Pulmonary Vascular Resistance (PVR) ≥ 5 Wood Unit (WU). Noninvasive methods have been proposed to measure PVR; however, their accuracy remains low. Right Ventricle (RV) - Pulmonary Artery (PA) coupling is defined as the ability of the RV to adapt to high-resistance conditions. Tricuspid Annular Plane Systolic Excursion (TAPSE)/estimated pulmonary artery systolic pressure (ePASP) calculation using echocardiography is a noninvasive technique that has been proposed as a surrogate equation to evaluate RV-PA coupling. Currently, no research has demonstrated a relationship between RV-PA coupling and PVR in patients with ASD. METHODS: The study participants were consecutive eligible patients with ASD who underwent right heart catheterization (RHC) and echocardiography at Hasan Sadikin General Hospital, Bandung. Both the procedures were performed on the same day. RV-PA Coupling, defined as TAPSE/ePASP > 0.31, was assessed using echocardiography. The PVR was calculated during RHC using the indirect Fick method. RESULTS: There were 58 patients with ASD underwent RHC and echocardiography. Among them, 18 had RV/PA Coupling and 40 had RV/PA Uncoupling. The PVR values were significantly different between the two groups (p = 0.000). Correlation test between TAPSE/ePASP with PVR showed moderate negative correlation (r= -0.502, p = 0.001). TAPSE/ePASP ≤ 0.34 is the cutoff point to predict PVR > 5 WU with sensitivity of 91.7% and specificity 63.6%. CONCLUSION: This study showed a moderate negative correlation between TAPSE/ePASP and PVR. TAPSE/ePASP ≤ 0.34 could predict PVR > 5 WU with good sensitivity.

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BACKGROUND: Balloon sizing (BS) has been used for device size selection in percutaneous atrial septal defect (ASD) closure. Due to its limitations, alternative imaging techniques like three-dimensional transesophageal echocardiography (3D-TEE) are valuable for guiding ASD device size selection during ASD closure procedures. The purpose of this study was to compare ASD sizing using measurements obtained from 3D-TEE to those utilizing the standard balloon sizing method. METHODS: We identified 53 patients with single secundum type ASD without PFO who underwent percutaneous closure at the Tehran Heart Center between 2019 and 2022. Balloon sizing was performed in all patients with the stop-flow technique, and the choice of device size was determined based on the sizing derived from BS. 3D-TEE imaging was performed before the intervention, and the ASD shape and quality of ASD rims were assessed. RESULTS: Among the 53 patients who underwent single ASD device closure, multiple 3D TEE measurements significantly correlated with balloon sizing results. This included defect area, perimeter, and diameter obtained from 3D-TEE images multi-planar reconstruction. ASD perimeter detected by 3D TEE had the best correlation with BS results. When divided by the shape of ASD, there was no significant difference between our 3D-images data and BS in round or oval-shaped ASDs. CONCLUSION: The 3D-TEE study is reliable for assessing ASD configurational characteristics in percutaneous device closure candidates. 3D-TEE has the potential to accurately determine the appropriate device size and reduce complications, costs, and procedural duration. Further research is needed to validate these findings and establish the role of 3D-TEE measurements in guiding the best treatment decisions for ASD closure.

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Double outlet right atrium is a rare congenital cardiac abnormality that has been previously reported in humans and cats, but not in dogs. A double outlet right atrium is typically characterized by the presence of a leftward deviation of the interatrial septum and atrial septal defect. Therefore, the right atrium drains into both ventricles. The unique features consistent with double outlet right atrium were identified by transthoracic echocardiography and computed tomography in a puppy. This case report describes the clinical, echocardiographic, and tomographic findings of a five-month-old Cocker Spaniel diagnosed with this rare congenital abnormality.

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CLINICAL DATA: Female, seven years old, referred to our service complaining about congestive heart failure symptoms due to mitral valve regurgitation and atrial septal defect. Technical description: Echocardiographic findings compatible with Barlow's disease and atrial septal defect, ostium secundum type. OPERATION: She was submitted to mitral valvuloplasty with chordal shortening and prosthetic posterior ring (Gregori-Braile®) along with patch atrioseptoplasty. COMMENTS: Mitral valve regurgitation is a rare congenital heart disease and Barlow's disease is probably rarer. Mitral valve repair is the treatment of choice.

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The concomitant large atrial septal defect closure, endocardial biatrial cryoablation and tricuspid valve replacement with mitral homograft in a patient with adult congenital heart disease is presented. Because of the severely dilated right ventricle and leaflet tenting, tricuspid valve replacement was considered. The patient refused both mechanical and stented biological prosthesis due to personal beliefs, therefore, the alternative valve substitute was proposed. Relevant decision-making process, preoperative diagnostic work-up and surgical technique are highlighted with satisfactory outcomes.

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BACKGROUND: Transcatheter correction of sinus venosus defects (SVDs) using balloon-mounted covered stents provides an attractive surgical alternative. Surgery may be complicated by superior vena caval or right upper pulmonary vein (RUPV) stenosis, sinus nodal dysfunction, and residual additional pulmonary veins. OBJECTIVES: Being a new intervention, technical modifications would simplify the procedure, improve universal applicability, and reduce or tackle complications. METHODS: Patients were included if balloon interrogation of cavoatrial junction confirmed closure of SVD and redirected RUPV to the left atrium. A single-center experience was analyzed to summarize the procedural modifications over 8 years. Transesophageal echocardiogram (TEE) on follow-up was done to identify residual shunt, RUPV flows, and stent thrombosis. RESULTS: A total of 100 patients including 9 children with a median age of 35 years (range, 4-69 years) underwent SVD closure after balloon interrogation. Among 57 patients balloon interrogated in the first 5 years, 70% underwent transcatheter closure, with 2 failures. RUPV occlusion caused the exclusions. Inclusions improved to 94% among the subsequent 65 balloon interrogations when RUPV protection was implemented, with 1 failure. Stent embolization caused the 3 failures warranting surgery. Recent modifications included limited transesophageal echocardiogram without anesthesia, avoiding venovenous circuit, interrogation with semicompliant balloons, trans-septal RUPV protection, overlapping stents to permit additional vein drainage to superior vena cava and tackle embolizations. There were no deaths. Minor complications included stent embolizations stabilized in catheterization laboratory in 2 patients, left innominate vein jailing in 2 patients, insignificant residual flows, and nonocclusive asymptomatic stent thrombosis in 4 patients. CONCLUSIONS: Procedural success was 97%. Recent modifications increased patient inclusions, decreased complications, and simplified the intervention.

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BACKGROUND: The accuracy of right ventricular (RV) quantification by three-dimensional echocardiography (3DE) has been reported mainly in patients with a normal right ventricle (RV). However, there are no data regarding the accuracy of 3DE in patients with a dilated RV, as in shunt diseases. In this study, we evaluated the accuracy of 3DE and that of volumetric (Vol) cardiac magnetic resonance (CMR) for assessment of RV and left ventricular (LV) stroke volume (SV) and the pulmonary (Qp)/systemic (Qs) blood flow ratio in patients with an atrial septal defect (ASD) using the two-dimensional phase contrast (2DPC) method as the gold standard. METHODS: We retrospectively investigated 83 patients with ASD who underwent transcatheter closure and clinically indicated CMR and 3DE examinations. The ratio Qp/Qs was calculated using RV and LV SV measured by full-volume volumetric 3DE (Vol-3DE) and CMR (Vol-CMR) and by two-dimensional pulsed Doppler quantification (2D-Dop); the parameters were compared using 2DPC-CMR as the gold standard. RESULTS: There was no significant difference in the Qp/Qs value between 2DPC-CMR and Vol-3DE (2.29 ± 0.70 vs 2.21 ± 0.63, P = .79) and 2D-Dop (vs 2.21 ± 0.65, P = 1.00); however, a significant difference was found between 2DPC-CMR and Vol-CMR (P < .001). The Qp/Qs value obtained using Vol-3DE showed the best correlation with 2DPC-CMR (r = 0.93, P < .001). The RV and LV SV values obtained by Vol-3DE showed the best correlation with 2DPC-CMR (RV SV, r = 0.82, P < .001; LV SV, r = 0.73, P < .001), although the absolute values were underestimated. CONCLUSION: Qp/Qs was more accurately evaluated by Vol-3DE than by Vol-CMR or 2D-Dop. Three-dimensional echocardiography assessment was feasible and reproducible even in a dilated RV.

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Transesophageal echocardiography (TEE) shows pericardial effusion and a gap between the left atrium and the aortic sinus by atrial septal defect occluder.

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The morphologic features of the multiple atrial septal defects assessed by TTE-based 3D imaging were similar to those by 3D-TEE. TTE-based 3D model had excellent visibility, allowing observation of 3D structure of the rims of the defects. It may be useful method for assessment of the multiple atrial septal defects.

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BACKGROUND: Recognition of right-to-left shunt is crucial in the work-up of patients with suspected patent foramen ovale (PFO) or atrial septal defect (ASD). While transesophageal echocardiography (TEE) remains the gold standard diagnostic tool for the anatomic assessment of PFO/ASD, transcranial Doppler (TCD) and contrast-enhanced transthoracic echocardiogram (CE-TTE) hold the promise of providing minimally invasive yet accurate clinical details. Their comparative accuracy remains however debated. METHODS: We conducted a retrospective observational study leveraging our extensive institutional experience with systematic TCD and CE-TTE in patients with suspected PFO/ASD. Several measures of diagnostic test accuracy were computed, with point estimates and 95% confidence intervals, when applicable. RESULTS: A total of 1358 patients were included, with age 48±14 years and 772 (58%) women. Tests were performed for diagnostic purposes in 797 (58.6%) and during follow-up in 740 (54.5%). A PFO was eventually diagnosed in 1038 (77.9%) patients, and an ASD in 60 (4.5%). Agreement between TCD and CE-TTE occurred in 1309 (85.2%) cases, with TCD yielding worse findings than CE-TTE in 91 (5.9%) patients, and vice versa in 137 (8.9%), yielding a Cohen kappa of 78.6% (95% CI: 76.3-81.1%) and a highly significant P value at McNemar test (P<0.001). After dichotomization, and using TCD as benchmark, CE-TTE yielded sensitivity 96.9%, specificity 95.1%, area under the curve 92.1%, and P=0.249. Similar findings were obtained when focusing only on diagnostic tests or follow-up ones (Cohen kappa respectively 74.0% [70.2-77.1%], P<0.001 and 80.3% [76.4-84.3%], P<0.001). Notably, Valsalva was necessary to disclose the presence of shunt during TCD in 487 (31.7%) patients and during CE-TTE in 482 (31.4%) cases. Finally, performance of TCD and CE-TTE in a subset of patients eventually undergoing TTE was quite similar. CONCLUSIONS: The diagnostic accuracy of CE-TTE appears favorable, and this imaging test may identify patients who may be missed if only TCD is used to screen patients with suspected PFO/ASD. Accordingly, CE-TTE is recommended as an adjunct diagnostic modality for all patients with a high pre-test probability of PFO/ASD and right-to-left shunt.

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