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Background: Transcatheter (TC) atrial septal defect (ASD) closure has been the mainstay of therapy for secundum-type ASDs for over 20 years., Aims: This nationwide cohort evaluated the long-term outcome of transcatheter-closed ASDs., Methods: The study enrolled every transcatheter ASD closure performed in Finland from 1999 to 2019. Five age, sex, and municipality-matched controls per ASD patient were gathered from the general population. The median follow-up period was 5.9 years (range 0-20.8). We used the hospital discharge register to gather all hospital visits and diagnoses. Closure complications and echocardiographic changes were collected from the electronic health records., Results: Transcatheter ASD closure was performed in 1000 patients (68.5% females) during the study period. The median (range) age at the time of the procedure was 37.9 (1.8-87.5) years. ASD patients had an increased risk for new-onset atrial fibrillation (RR 2.45, 95% CI: 1.84-3.25), migraine (RR 3.61, 95% CI: 2.54-5.14), ischemic heart disease (RR 1.73, 95% CI: 1.23-2.45), ventricular fibrillation/tachycardia (RR 3.54 (95% CI: 1.48-8.43) and AV conduction disorder (RR 3.60, 95% CI: 1.94-6.70) compared to the control cohort. Stroke risk was not increased (RR 1.36, 95% CI: 0.91-2.03). Adverse events occurred in 6.3% ( n  = 63) of the patients, including four erosions and ten device embolizations., Conclusion: After TC closure of ASD, patients had a higher risk of new-onset atrial fibrillation and migraine than controls without ASD . As novel findings, we found an increased risk for ischemic heart disease, AV conduction disorders, and ventricular fibrillation/tachycardia.Key messagesEven though patients have an excellent overall prognosis after percutaneous ASD closure, the increased incidence of major comorbidities like atrial fibrillation and heart failure prompts more thorough lifelong follow-up.This study's novel findings revealed the increased risk for ischemic heart disease, AV conduction disorders, or ventricular tachycardia/fibrillation during the follow-up.Major complications after the closure are rare; erosion is seen in 0.4% of the patients and embolization in 1.0% of the patients.

Background: Transcatheter closure of atrial septal defect (ASD) has become the treatment of choice for most patients. About 5% of them suffer from transient headache episodes (THE) after the procedure, whose etiology is unclear., Aims: To evaluate risk factors for THE occurrence after transcatheter closure of ASD in the pediatric population., Methods: Eight hundred and forty patients, after transcatheter ASD closure with nitinol devices, from a single center, were included in retrospective analysis. THE was defined as occurring up to 24 hours after the procedure. A logistic regression model including age, weight, ASD diameter, device size, presence of nitinol coating on the device, fluoroscopy time, application of balloon calibration, device oversizing, and residual shunt after 24 hours was created to evaluate risk factors for THE occurrence., Results: There were 40 patients with THE (4.8%), 70% female and 30% male. The median age was 13 (7.35-16) years. In patients with headache, balloon calibration (BC) was performed more frequently (82.5% vs. 43.3%; P <0.001). The balloon waist median (interquartile range [IQR]), 19 (16-22) mm vs. 15 mm (12-18) mm (P <0.001), and device size median (IQR), 18 (13.5-22) mm vs. 14 (11-17) mm (P <0.001) were larger, and residual shunt after 24 hours (12.5% vs. 4.9%; P = 0.03) and a year (7.5 vs. 1.0%; P <0.001) were more frequent. ASD size and the prevalence of double/multiple ASD were similar in both groups. Age, BC application, no nickel release protection, duration of fluoroscopy, and device oversizing were predictors of THE (P <0.001)., Conclusions: BC during percutaneous ASD closure and the lack of a protective layer against nickel release on the device are risk factors for headache occurrence in the early postprocedural period.

Objectives: To introduce a novel method of direct iatrogenic atrial septal defect (iASD) closure through the MitraClip steerable guide catheter (SGC)., Background: MitraClip implantation requires transseptal puncture and the creation of an iASD. There are relatively rare instances, such as hemodynamically significant shunting or concerns for embolus, where iASD must be closed during index procedure. In these instances, it may be beneficial to not give up access to left atrium., Methods: We retrospectively reviewed all iASD closures during MitraClip implantation at our institution from 2015 to 2020. Cases where an ASD occluder was deployed directly through SGC were included., Results: Eleven patients had immediate iASD closure through the SGC. Indications for using this method included concern for paradoxical embolus, large defect size and/or significant shunting. Closure device sizes ranged from 8 to 22 mm. Mean time from removal of clip delivery system to occlusion of iASD was 14.6 minutes. There were no procedural complications related to iASD closure using this method., Conclusion: Closure of iASD intra-procedurally directly through transseptal guide sheath via the method described was safe and allowed for continuous left atrium access.

Objectives: To assess the safety and efficacy of the Amplatzer Septal Occluder in the closure of secundum type atrial septal defects., Background: The Amplatzer Septal Occluder (ASO; Abbott, St. Paul, MN) is an FDA-approved device for percutaneous closure of secundum type atrial septal defects (ASD). Previous small cohort trials have shown a favorable safety and technical efficacy profile., Methods: We conducted a systemic review and meta-analysis of all prospective case series and controlled trials that evaluated the ASO's safety and implant efficacy. The primary endpoint was the technical success rate of implantations. Secondary outcomes included proportions of arrhythmias and embolism specific-adverse events., Results: We included a total of 12 studies with 2972 patients. The ratio of device implantation was 2:1 by sex [female: male]. Pooled technical success rate of implantation was 98% (95% CI: 0.968-0.990, P < 0.01). The cumulative adverse event rate was 5.1% (95% CI: 0.035-0.068, P < 0.01), which included arrhythmia and embolism specific adverse event rates of 1.8% (95% CI: 0.007-0.032, P < 0.01) and 0.7% (95% CI: 0.002-0.013, P < 0.01), respectively. Sensitivity analysis did not significantly affect pooled outcomes for success rate and adverse events; both forest plot and Begg's and Egger's regression tests supported symmetricity., Conclusion: A high likelihood of technical success can be expected when implanting the ASO in secundum type ASDs. Adverse event rates are expected for one in twenty patients, and thus, our results support the safe use of ASO in secundum type ASDs closure., Condensed Abstract: The AMPLATZER Septal Occluder is an FDA-approved device for percutaneous closure of secundum type atrial septal defects (ASD). We conducted a systemic review and meta-analysis of all prospective case series and controlled trials that evaluated the ASO's safety and implant efficacy. We included a total of 12 studies with 2972 patients. Pooled technical success rate of implantation was 98% (P < 0.01). The cumulative adverse event rate was 5.1% (P < 0.01), 1.8% (P < 0.01) rate of arrhythmias, and 0.7% (P < 0.01) rate of embolisms. A high likelihood of technical success can be expected with a low rate of adverse events., Competing Interests: Declaration of competing interest The authors report no conflicts of interest regarding the content herein., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objectives: To investigate prevalence of iatrogenic atrial septal defects (iASDs) after mitral valve (MV) transcatheter edge-to-edge repair (TEER) with the MitraClip in the long-term follow-up., Background: MV TEER requires transseptal puncture using a large 22 F sheath. Prevalence, impact and haemodynamic effects of these iASDs remain unknown in the long-term follow-up., Methods: This prospective study enrolled patients who had undergone first-time TEER at our university hospital between January 2017 and June 2018 for a clinical long-term follow-up study. Prevalence of iASD was investigated 12 months post-TEER using transoesophageal echocardiography (TEE). Study protocol further consisted of transthoracic echocardiography (TTE) and exercise testing. Incidence of all-cause death was compared 12 months post-TEE follow-up. This study was approved by local ethics committee., Results: 48 patients participated in clinical follow-up examinations. Median time between TEER and clinical follow-up examination (TEE, TTE, exercise testing) was 19.5 (IQR: 7.0) months after TEER. Persistent iASD was found in 41.7% of patients. TEER was found to be equally effective in reducing MR and clinical symptoms in both groups at baseline as well as follow-up. Procedural fluoroscopy and device times were significantly longer in the iASD group. MR reduction and functional status (New York Heart Association Class, 6 Minute Walking Test distance) were similar in both groups. Nevertheless, a significant decrease in systolic pulmonary artery pressure (sPAP) and significantly smaller atrial diameters were observed in patients with iASD at follow-up., Conclusion: Prevalence of iASDs after TEER in long-term follow-up was about 41%. Although a significant reduction of sPAP and better left atrial reverse remodelling were accomplished in patients with iASD, clinical impact appears low. Manipulation at the atrial septum might play a key role in creating persisting iASD., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Percutaneous mitral valve repair (PMVR) requires transseptal puncture and results in iatrogenic atrial septal defect (iASD). The impact of persistent iASD was previously investigated. However, data were diverse and inconclusive. 53 patients who underwent MITRACLIP were retrospectively included. Based on the presence of iASD in transesophageal echocardiography (TEE) after 6 months, patients were divided in two groups (iASD group vs. non-iASD group). Impact of iASD on outcome at 6 months and at two years was evaluated. Persistent iASD was detected in 62% of patients. Independent predictors for persistent iASD were female gender and reduced left ventricular ejection fraction. At 6-month follow-up, there was no difference in reduction of NYHA class (ΔNYHA = 1.3 ± 1 in iASD group vs. 0.9 ± 1 in non-iASD group, p = 0.171). There was a significant difference in right ventricular end diastolic diameter (RVEDd) (42 ± 8 mm in iASD-group vs. 39 ± 4 mm in non-iASD group, p = 0.047). However, right ventricular systolic function (TAPSE) (14 ± 7 mm in iASD group vs. 16 ± 8 mm in non-iASD group, p = 0.176) and right ventricular systolic pressure (RVSP) (40 ± 12 mmHg in iASD group vs. 35 ± 10 mmHg in non-iASD group, p = 0.136) were still comparable between both groups. At 2 years follow-up, there was no significant difference regarding rate of rehospitalization (24% vs 15%, p = 0.425) or mortality (12% vs 10%, p = 0.941) between both groups. Incidence of persistent iASD after MITRACLIP is markedly high. Despite the increase in right ventricular diameter in patients with persistent iASD, these patients were not clinically compromised compared to patients without persistent iASD.

Percutaneous MitraClip intervention for treatment of severe mitral regurgitation in high surgical risk patients requires large-diameter transseptal sheaths that can result in iatrogenic atrial septal defect (iASD), and its prevalence is higher compared with non-MitraClip procedures. This iASD is not routinely closed because the possible consequences are still not fully understood. However, we believe it is important to identify patients who may benefit from its closure immediately after the procedure to prevent hemodynamic deterioration and long-term negative clinical outcomes. We describe our experience with 2 patients who required iASD closure after MitraClip procedure due to right-to-left shunt resulting from increase in right heart pressures.

Objectives: The aim of this study was to investigate the incidence, characteristics, hemodynamic conditions, and clinical significance of right-to-left (R-L) shunt through an iatrogenic atrial septal defect (iASD) after the MitraClip procedure., Background: R-L shunt through an iASD after the MitraClip procedure has not been well investigated., Methods: From 2014 to 2017, 385 consecutive patients with mitral regurgitation underwent the MitraClip procedure. iASD was assessed using intraprocedural transesophageal echocardiography. Right and left heart catheterization was used to assess the hemodynamic status of patients. All patients provided written informed consent for the procedure. All data for this study were collected from an established interventional cardiology laboratory database approved by the Cedars-Sinai Medical Center Institutional Review Board., Results: R-L shunt was observed in 20 patients (5%). In 7 of these patients (35%), R-L shunt was accompanied by acute deoxygenation. Prevalence of severe tricuspid regurgitation (55% vs. 20%; p = 0.001), serum B-type natriuretic peptide (664 pg/ml [434 to 1,169 pg/ml] vs. 400 pg/ml [195 to 699 pg/ml]; p = 0.006), mean pulmonary artery pressure (38 mm Hg [34 to 45 mm Hg] vs. 29 mm Hg [22 to 37 mm Hg]; p < 0.001), and right atrial pressure (19 mm Hg [13 to 20 mm Hg] vs. 10 mm Hg [7 to 14 mm Hg]; p < 0.001) were significantly higher in patients with R-L shunt than in those with left-to-right shunt. Patients with R-L shunt also showed a more prominent reduction in the left atrial V-wave and mean pressure from baseline to post-procedure compared with those with left-to-right shunt (-22.8 ± 2.6 mm Hg vs. -11.8 ± 0.9 mm Hg [p = 0.002] and -7.9 ± 0.8 mm Hg vs. -4.0 ± 0.4 mm Hg [p = 0.003], respectively)., Conclusions: R-L shunt through an iASD was observed in 5% of patients who underwent the MitraClip procedure and in one-third of patients with R-L shunt presented acute deoxygenation. Elevated right atrial pressure concomitant with pulmonary hypertension and significant reduction in left atrial pressure after MitraClip deployment were associated with R-L shunt., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

In Ebstein's anomaly, percutaneous atrial septal defect (ASD) closure for the treatment of hypoxemia due to a right-to-left interatrial shunt remains controversial. We report the case of a 40-year-old woman with Ebstein's anomaly who developed cyanosis and shortness of breath on exercise. Her symptoms improved after percutaneous ASD closure and her clinical course has been good during follow-up. The balloon ASD occlusion test, combined with dobutamine stimulation before the procedure, is useful to confirm treatment indication. A prior electrophysiological evaluation is also important because Ebstein's anomaly is often complicated by atrioventricular recurrent tachycardia.

The atrial septum enables efficient oxygen transport by separating the systemic and pulmonary venous blood returning to the heart. Only in placental mammals will the atrial septum form by the coming-together of the septum primum and the septum secundum. In up to one of four placental mammals, this complex morphogenesis is incomplete and yields patent foramen ovale. The incidence of incomplete atrial septum is unknown for groups with the septum primum only, such as birds and reptiles. We found a low incidence of incomplete atrial septum in 11 species of bird (0% of specimens) and 13 species of reptiles (3% of specimens). In reptiles, there was a trabecular interface between the atrial septum and the atrial epicardium which was without a clear boundary between left and right atrial cavities. In developing reptiles (four squamates and one crocodylian), the septum primum initiated as a sheet that acquired perforations and the trabecular interface developed late. We conclude that atrial septation from the septum primum only results in a low incidence of incompleteness. In reptiles, the atrial septum and atrial wall develop a trabecular interface, but previous studies on atrial hemodynamics suggest this interface has a very limited capacity for shunting., (© 2019 The Authors. Evolution & Development published by Wiley Periodicals, Inc.)

Purpose: Iatrogenic atrial septal defect (IASD) after catheter ablation (CA) for atrial fibrillation (AF) due to transseptal puncture (TSP) can occur. The aim of this prospective study was to describe the incidence of IASD and to detect any cerebrovascular accident (CVA) after radiofrequency (RF) and cryoballoon (CB) CA., Methods: Between July 2014 and September 2016, 94 patients (pts) (RF; 48, CB; 46, 30 (31.9%) women, mean age = 60 ± 9.7 years) with paroxysmal AF were enrolled who underwent CA procedure for the first time. During RF ablation a single (n = 30, 62.5%) or double (n = 18, 37.5%) TSP was performed. Transoesophageal echocardiography before the procedure and at the 3-month and 12-month follow-up (FU) was accomplished. During the FU period, we evaluated the occurrence of any postprocedural CVA., Results: At the 3-month FU, IASD was detected in 17/94 (18.1%) pts; in 9/48 (18.8%) pts in the RF while in 8/46 (17.4%) pts in the CB group (p = 0.866), all of them with left-to-right shunt. In the RF group, 6/30 (20%) pts with a single TSP while 3/18 (16.7%) pts in the double TSP group had IASD (p = 0.780). 14/17 (82.4%) IASDs showed high spontaneous closure rate at the 12-month FU. None of the pts died or suffered from CVA., Conclusion: Persistent IASD can occur rather frequently following AF CA. No significant difference was observed between the RF and CB techniques concerning the presence of IASD at 3-month. IASDs showed a high spontaneous closure rate. No cerebral thromboembolic event was observed in the 12-month FU period.

BACKGROUND Catheter ablation for atrial fibrillation is an important therapeutic intervention. One of the most frequent complications of this procedure is vascular issues including arteriovenous fistula. Iatrogenic atrial septal defect (IASD) has been reported as a complication of transseptal puncture; however, no data are available demonstrating any coexistent of arteriovenous fistula with IASD. CASE REPORT A 61-year-old female patient was admitted to our center for catheter ablation for persistent atrial fibrillation. Her past medical history was significant for cryoballoon ablation for atrial fibrillation in 2015, which was subsequently complicated by hematoma and arteriovenous fistula at puncture site. After general surgery consultation, the patient was qualified for conservative treatment. To exclude left atrial thrombus before redo procedure, transesophageal echocardiography was performed which visualized the presence of 9-mm atrial septal defect with left-to-right shunting, detecting right-to-left shunting using Valsalva maneuver. No significant valvular abnormalities were identified. The next day, pulmonary vein isolation for atrial fibrillation was performed. One month later, a control transthoracic echocardiogram (TTE) revealed hemodynamic significant left-to-right shunting with Qp/Qs 2.0 and high probability of pulmonary hypertension. Vascular surgery for arteriovenous fistula was successfully performed in October 2018. Subsequent TTE, performed a month later, confirmed no left-to-right shunting and no signs of pulmonary hypertension or diminishment of the right atrium. CONCLUSIONS Vascular access during catheter ablation for atrial fibrillation may result in arteriovenous fistula. This condition might affect right atrium pressure leading to increased diameter of previous puncture site at the interatrial septum, causing IASD with significant shunting. In this group of patients, arteriovenous fistula should be treated as soon as possible.

Background: Persistent iatrogenic atrial septal defect (iASD) is a common but poorly characterized complication after cryoballoon (CB) pulmonary vein isolation (PVI) procedures. We therefore investigate its prevalence, evolution, risk factors, and clinical outcomes in a prospective longitudinal study., Methods: A total of 108 patients (41 women, mean age 57 ± 11.3) underwent CB PVI for AF. Serial transesophageal echocardiography (TEE) was performed 9 months and then annually until 6 years after the procedure to study the characteristics of persistent iASD., Results: Persistent iASD occurred in 33 (30.6%) patients 9 months after CB PVI. Spontaneous closure of iASD was found in 6 (22.2%) and 3 (15.8%) patients 2 and 3 years after the procedures, respectively. No spontaneous closure was observed on 4, 5, and 6-year TEE follow-up. The projected long-term persistence rate of iASD after CB PVI was therefore 20% (30.6% × 0.778 × 0.842). Using multivariate logistic regression, a higher number of cryoapplications (≥ 2 minutes) was the only independent predictor of persistent iASD 9 months after CB PVI (odds ratio [OR] 1.207; 95% confidence interval [CI], 1.033-1.411, P = 0.018). Two (1.9%) patients with significantly larger iASD size than the others (long diameter 12.6 ± 0.8 vs 3.7 ± 1.5 mm, P < 0.001; short diameter 10.9 ± 0.2 vs 3 ± 1.1 mm, P < 0.001) required percutaneous closure because of exertional dyspnea and right ventricular enlargement. Over 129.7 patient-years follow-up, during which iASD persisted, there was no occurrence of neurologic events., Conclusions: Approximately one fifth of patients undergoing CB PVI will have permanently persistent iASD. Patients with defect sizes of greater than 10 mm may need percutaneous closure due to significant left-to-right shunting., (Copyright © 2018 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

A 75 year-old female with symptomatic severe mitral regurgitation of a bio-prosthetic valve secondary to valve leaflet perforation due to endocarditis underwent a mitral valve in valve replacement in an existing Hancock 27 mm valve using a trans-femoral/trans-septal approach. The procedure was complicated by an iatrogenic atrial septal defect post trans-septal puncture causing a severe right-to-left shunt due, possibly, to the combination of severe pulmonary hypertension and moderate tricuspid regurgitation the patient had suffered from at base line. Once the sheath was removed an immediate desaturation was observed which required emergent closure with an AMPLATZER ASD Occluder device (St. Jude Medical/Abbott Structural, St. Paul, Minnesota, MN). This stabilized the patient and returned her blood oxygen levels to normal. The case brings forth a rare but important complication that may occur during trans-septal procedures especially when using large sheaths. It emphasizes the importance of proper echocardiographic and hemodynamic evaluation prior to such procedures in order to be prepared for such complications., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Left atrial dissection is an exceedingly rare complication of cardiac surgery, with an incidence of 0.16%-0.84%. We report the first case of interatrial dissection and hematoma in association with the MitraClip procedure. Hemodynamically stable patients can be managed conservatively, with echocardiographic imaging, often with resolution of the dissection over the course of weeks. Our patient remained hemodynamically stable and asymptomatic post operation; at 1-month follow-up, echocardiogram showed resolution of the interatrial septal dissection.

Persistent iatrogenic atrial septal defects (iASDs) can be observed after intervention requiring a left atria (LA) access, including pulmonary vein isolation (PVI) of atrial fibrillation (AF). We investigated the incidence of iASDs post-second-generation cryoballoon ablation and the pre-procedural predictors. Eighty-three paroxysmal AF patients underwent PVI using second-generation cryoballoons. The LA was accessed with single 15-Fr steerable sheaths following a radiofrequency transseptal puncture, and the iASD was evaluated with transthoracic echocardiography (TTE), a median of 9.3 (7.1-13.3) months post-procedure. All patients underwent pre-procedural contrast-enhanced multi-detector computed tomography (CT) to evaluate the LA and PV anatomy. iASDs were detected by TTE in 7 (8.4%) patients, a median of 15.5 (6.8-17.3) months post-procedure. Patients with iASDs had significantly larger LA volumes and smaller atrial septal angles, defined as the angle between the atrial septum and sagittal line on the horizontal section at the height of the fossa ovalis, which could be the transseptal puncture site measured on CT, and more likely hypertension than those without. Multivariate analyses revealed that the atrial septal angle was the sole predictor of iASDs [odds ratio 0.764, 95% confidence interval (CI) 0.624-0.935, p = 0.009], and the optimal cut-off value was 57.5° (sensitivity 85.7%, specificity 88.2%, 95% CI 0.873-0.995, p < 0.0001). Patients with iASDs were asymptomatic and had no adverse clinical events during a 17.7 (14.4-25.8) month median follow-up. iASDs were still detectable in 8.4% of patients a median of 15.5 months after the second-generation CB ablation, and the atrial septal angle might aid in predicting persistent iASDs.

Purpose: Congenital atrial septal defect (ASD) is associated with increased morbidity, whereas little is known about the rate of spontaneous closure, associated clinical and echocardiographic parameters, or complications of iatrogenic atrial septal defect (iASD) beyond 1 year of follow-up. Persistent iASD after transseptal puncture for PVI has been described in up to 38% of small cohorts of patients in short-term follow-up after transseptal puncture. We sought to investigate the course of iASD after single transseptal puncture for first pulmonary vein isolation (PVI) with cryoballoon, along with possible risk factors for persistent iASD., Methods: After a first PVI with cryoballoon, 102 patients (64 ± 10 years, 64% male) underwent long-term clinical follow-up and comprehensive transthoracic and transesophageal echocardiographic study., Results: Prevalence of iASD after PVI was 37% after 2.9 (1.6-4.9) years. No clinical complications or deterioration of echocardiographic parameters were associated with iASD. Lower left atrial appendage flow velocity was associated with higher risk of persistence of iASD (3.5% for every 1 cm/s decrease, p = 0.002)., Conclusions: Despite a high rate of iASD after cryoballoon PVI in long-term follow-up, this was not associated with increased clinical complications. Lower LAA velocity was associated with higher risk of persistent iASD. Repeated routine echocardiographic follow-up may not be necessary in these patients.

This study examines the incidence of iatrogenic atrial septal defect (iASD) after the MitraClip procedure and its influence on echocardiographic and clinical outcomes. We examined 96 patients who underwent a successful MitraClip procedure and who also had baseline and 1-year postprocedure transthoracic echocardiograms. At 1-year follow-up, iASD were observed in 24% of cases. Compared with the patients without iASD, the patients with iASD had a larger right atrium and greater severity of tricuspid regurgitation (TR) at baseline. After the MitraClip procedure, mitral regurgitation lessened significantly in both groups. Although right atrial area and right ventricular diameters increased significantly in patients with iASD (25.3 ± 8.0 to 28.3 ± 9.5 cm 2 , 39.7 ± 7.1 to 42.2 ± 8.1 mm, p <0.05 for both comparisons), these variables did not change in patients without iASD. In addition, patients with iASD had worse TR at follow-up. The incidence of stroke was comparable between the 2 groups during 1-year follow-up (4.3% vs 4.1%). However, patients with iASD had a markedly higher re-hospitalization rate for heart failure (26% vs 2.7%, p <0.05). In conclusion, iASD occurred in 24% of patients who underwent the MitraClip therapy and the presence of iASD was associated with right-sided heart enlargement, worse TR, and a higher re-hospitalization rate for heart failure., (Copyright © 2017. Published by Elsevier Inc.)

Rationale: Iatrogenic atrial septal defects caused by cardiac surgery are rare complications that are traditionally repaired through reoperations; unfortunately, reoperations are accompanied by high risk and trauma., Patient Concerns: Herein, we report a rare case of a huge atrial septal defect after mitral and aortic mechanical valve replacement., Diagnoses: Transesophageal echocardiography revealed a 20 × 33 mm atrial septal defect with a mainly left-to-right shunt and bidirectional shunt., Interventions: The defect was successfully occluded using a Shape Memory septal occlude with a waist diameter of 42 mm., Outcomes: At follow-up 6 months after, the patient's symptoms were remarkably relieved and chest radiograph showed obvious improvement of the pulmonary congestion., Lessons: Percutaneous device treatment can be used as an alternative to surgery in iatrogenic atrial septal defects if the anatomical condition of the septal defect is appropriate for transcatheter closure., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Background: The pattern and risk factors for congenital heart diseases (CHD) in children with Down syndrome (DS) vary over time., Objectives: To update knowledge of the prevalence, types, trends and associated factors for CHD in children with DS in the Egyptian Delta., Design: A retrospective hospital record-based descriptive study., Setting: A tertiary care center in Mansoura, Egypt during a period of 14 years from 2003 up to 2016., Patients and Methods: We studied children with genetically proven DS. Relevant sociodemographic factors, medical history, clinical examination, karyotype and echocardiographic data were statistically analyzed., Main Outcome Measures: Prevalence, types and risk factors of CHD in DS., Results: The prevalence of overall, isolated and multiple CHD in 1720 children with DS were 36.9%, 29% and 8%, respectively. Isolated defects accounted for 78.4% of all CHD. Ventricular septal defect, atrioventricular septal defect and atrial septal defect were the most frequent isolated defects. There was a downward trend in the prevalence of overall CHD (from 56.2% to 25.0%) and isolated CHD (from 56.2% to 19.8%). The logistic regression model predicted 65.7% of CHD and revealed that passive maternal smoking, lack of folic acid/multivitamin supplementation and parental consanguinity were the independent predictors of CHD in children with DS with adjusted odds ratios of 1.9, 1.8 and 1.9, respectively., Conclusion: More than one-third of children with DS have CHD with ventricular septal defect, which is the most common. Avoidance of passive maternal smoking and consanguineous marriage together with maternal folic acid supplementation could contribute to further reduction of CHD in children with DS., Limitations: Single-center study and retrospective.

In recent years, the transseptal puncture approach has enabled passage of increasingly large and complex devices into the left atrium. Traditional tools remain effective in creating and dilating the initial puncture, with an acceptable safety profile. Even for skilled operators, the procedure is technically demanding and requires sound understanding of atrial anatomy. Intracardiac echocardiography is useful in cases of previous septal repair, poorly defined fossa ovalis anatomy or when considering patent foramen ovale portal crossing. Iatrogenic atrial septal defect (iASD) is the most commonly encountered long-term complication and there is increasing evidence that larger devices are leading to symptomatic defects. The size of the sheath crossing the septum is the strongest predictor of iASD formation but other factors such as longer procedure times, significant catheter manipulation and high pulmonary pressures also contribute. Transcatheter mitral valve repair involves the use of large 22 Fr catheters which carry alarmingly high rates of defect persistence with precipitation of symptoms and possible influence on mortality. Long-term follow up data, particularly beyond the 12-month period are lacking and resultantly, evidence to guide management is sparse. Refinements of conventional instruments, as well as innovations to puncture the septum without mechanical pressure, herald a progressively safer future for the transseptal technique., (Copyright © 2017 Elsevier B.V. All rights reserved.)

We report a case of chimpanzee trisomy 22 in a captive-born female. Because chromosome 22 in great apes is homologous to human chromosome 21, the present case is analogous to human trisomy 21, also called Down syndrome. The chimpanzee in the present case experienced retarded growth; infantile cataract and vision problems, including nystagmus, strabismus, and keratoconus; congenital atrial septal defect; and hypodontia. All of these symptoms are common in human Down syndrome. This case was the second reported case of trisomy 22 in the chimpanzee. The chimpanzee in our case became blind by 7 years old, making social life with other chimpanzees difficult, but opportunities to interact with other conspecific individuals have been offered routinely. We believe that providing her with the best care over the course of her life will be essential.

Purpose: Pulmonary vein isolation using cryoballoon ablation is an established approach to treating atrial fibrillation. The procedure involves insertion of a 15-Fr sheath into the left atrium across the interatrial septum. This creates an iatrogenic atrial septal defect, which may have important long-term clinical relevance, especially in younger patients. We sought to determine the long-term incidence of these defects and determine the direction of shunt using contrast transesophageal echocardiography., Methods: Individuals who had undergone a single pulmonary vein isolation procedure were invited to attend for transesophageal echocardiography (TOE). Patients who had undergone more than one procedure involving puncture of the interatrial septum were excluded. The interatrial septum was interrogated using two-dimensional imaging, color flow Doppler, and microbubble contrast study., Results: A total of 27 patients were recruited with a median follow-up time of 553 days from pulmonary vein isolation to TOE. Seven patients had persistent iatrogenic atrial septal defects with three demonstrating right to left shunt either at rest or with Valsalva. There were no reported adverse events during the study period., Conclusions: Persistent iatrogenic atrial septal defects are relatively common following cryoballoon ablation procedures. Right to left shunting can be observed using microbubble contrast in a subset of patients with iatrogenic atrial septal defect (iASD). Further studies that longitudinally assess shunt fraction, pulmonary artery pressure, and the incidence of paradoxical embolism are needed to better understand the clinical impacts of such defects.

Cardiac septal defects are known complications to blunt chest trauma. The incidence of a traumatic isolated atrial septal defect is unknown and the concurrent occurrence of nonlethal ventricular and atrial septal defects has not been reported. A healthy male sustained violent blunt chest trauma resulting in traumatic cardiac septal disruption in the atrium and ventricle. The defects were detected by echocardiography within 14 hours of the accident. The extent of damage was confirmed at the time of surgical repair. The patient recovered uneventfully. The diagnosis and management of concurrent ASD and VSD is similar to single septal injury., (© 2014, Wiley Periodicals, Inc.)

Iatrogenic erosion of the septum primum after foramen ovale closure is an anecdotal event. We report the case of a 39-year-old woman admitted to our institution for multifocal cryptogenic cerebral ischemia and a patent foramen ovale (PFO) associated with an aneurysm of the septum primum. The patient underwent percutaneous closure of the PFO with an Amplatzer PFO Occluder device. At the 6-months follow up, the device was in the right position, but a jagged defect of the septum primum and evidence of significant left-to-right shunting was detected. The atrial septal defect was then repaired by a surgical approach. Although this event is not life-threatening, it should be considered as a therapeutic pitfall, resulting in a risk of paradoxical embolism recurrences and long-term hemodynamic impairment. © 2013 Wiley Periodicals, Inc., (© 2013 Wiley Periodicals, Inc.)

We report a case of a successful heart transplantation from a donor after multiple trauma. A traumatic atrial septal defect was repaired, and the postoperative course was uneventful., (© 2014 Wiley Periodicals, Inc.)

Percutaneous mitral valve repair using the MitraClip device has become a therapeutic alternative for high surgical risk patients with symptomatic mitral regurgitation. The procedure involves transseptal puncture and results in a new atrial septal defect (ASD) after withdrawal of the 22Fr guiding catheter. The functional effect of the new ASD is not defined. In 28 patients with symptomatic mitral regurgitation undergoing percutaneous mitral valve repair using the MitraClip device, 3-dimensional transesophageal echocardiography was used to measure by direct en face imaging the area of the new ASD. Analysis of the velocity-time integral (VTI) across the ASD after withdrawal of the guiding catheter allowed calculation of the shunt volume. Diastolic VTI of the mitral flow was determined before and after withdrawal of the guiding catheter to determine left ventricular inflow changes. Invasive left atrial pressure measurements were obtained during withdrawal of the guiding catheter. Regurgitant volume was reduced from 86±21 ml/beat before intervention to 43±22 ml/beat after intervention. The new ASD had an area of 0.19 cm2, 44% of the area of the 22Fr guiding catheter. Considering the VTI across the septal defect of 72±26 cm/s, the left-to-right atrial shunt volume was calculated to be 14±6 ml/beat. The diastolic forward flow across the mitral valve was reduced by 13±6 ml/beat immediately after withdrawal of the MitraClip guiding catheter. Mean left atrial pressure was reduced from 17±8 mm Hg with the guiding catheter still in the left atrium to 15±8 mm Hg after withdrawal of the guiding catheter. In conclusion, the creation of a new ASD as consequence of the large-diameter MitraClip guiding catheter results in volume and pressure relief of the left atrium. This contributes to the immediate hemodynamic changes implemented by the MitraClip procedure., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Aims: We sought to assess the hemodynamic sequel and the therapy adopted in patients treated with MitraClip system, who experienced a persistent interatrial communication (IAC) after the procedure., Methods: From January 2012 to March 2013, 28 consecutive patients (mean age 74 +/- 8 years) underwent transcatheter MitraClip repair, as part of an ongoing prospective study to assess the IAC., Results: Acute procedural success was 100% and a 30-days survival of 97% was reported. Immediately after the procedure, a residual IAC was present in all patients with an average diameter of 0.51 +/- 0.39 cm. At 30 days, IAC was detected in 81% of patients with a diameter of 0.45 +/- 0.31 cm (P = 0.53). To date, three patients developed different hemodynamic impairment secondary to the residual IAC, requiring percutaneous closure of the defect., Conclusion: Anatomic and hemodynamic criteria for early recognition of harmful residual IAC have to be identified, in order to select those patients who are benefited the most from atrial septal defect closure.

The aim of this study was to assess the risk factors in the origin of lethal or surgically corrected isolated atrial septal defect secundum. The population-based Hungarian Case-Control Surveillance of Congenital Abnormalities (conducted between 1980 and 1996) comprised 472 atrial septal defect secundum cases, 678 matched controls and 38,151 available controls without any defects; in addition, 21,022 malformed controls with other isolated defects. Medically recorded chronic disorders in the prenatal maternity logbook were evaluated, while acute maternal diseases, drug treatments and pregnancy supplements were analyzed on the basis of both prospective medically recorded data and retrospective maternal information. Acute pelvic inflammatory disease, paroxysmal supraventricular tachycardia and phenolphthalein treatment due to severe constipation of mothers were shown to contribute to the development of atrial septal defect secundum of their children. High doses of folic acid in early pregnancy had positively influenced a minor part of isolated atrial septal defect secundum in foetuses. In conclusion, the obvious genetic predisposition for atrial septal defect secundum is connected with maternal paroxysmal supraventricular tachycardia and triggered by acute pelvic inflammatory diseases and phenolphthalein treatment, while the manifestation of atrial septal defect secundum can be reduced by high doses of folic acid supplementation in early pregnancy.

We report on a combination of congenital malformations in a mother and her fetus harboring a heterozygous deletion encompassing the TBX5 and TBX3 genes, which are disease-causing in Holt-Oram and ulnar-mammary syndromes, respectively. This contiguous gene syndrome is reminiscent of Okihiro syndrome and emphasizes the importance of array-CGH as a diagnostic tool in atypical syndromic presentations with intrafamilial variability., (Copyright © 2013 Wiley Periodicals, Inc.)

Objectives: In general, epidemiological studies have evaluated cases with congenital cardiovascular abnormalities together. The aim of this study is to describe the birth outcomes of cases with isolated/single atrial septal defect type II (ASD-II, i.e. only a fossa ovalis defect) after surgical correction or lethal outcome in the light of maternal sociodemographic data., Design: Comparison of birth outcomes and maternal characteristics of cases with ASD-II and controls without defect., Setting: The population-based Hungarian Case-Control Surveillance of Congenital Abnormalities., Population: Hungarian newborn infants with or without ASD-II., Methods: Medically recorded birth outcomes, maternal age and birth order were evaluated. Marital and employment status was based on maternal information. The lifestyle factors were analyzed in a subsample of mothers visited at home based on a personal interview with mothers and their close relatives, and the family consensus was accepted., Main Outcome Measures: Mean gestational age at delivery and birthweight, rate of preterm birth and low birthweight, maternal age, birth order, marital and employment status., Results: The evaluation of 471 cases with ASD-II and 38,151 controls without any defects showed a female excess in cases with ASD-II, having shorter gestational age and lower mean birthweight, and thus a higher rate of preterm births and low birthweight., Conclusions: Intrauterine growth restriction and shorter gestational age were found in cases with ASD-II, particularly in female children. These factors may have a general developmental process in which there was not closure of the foramen ovale, thus echocardiographic screening of these babies might be of value., (© 2012 The Authors Acta Obstetricia et Gynecologica Scandinavica © 2012 Nordic Federation of Societies of Obstetrics and Gynecology.)

Gerbode defect, a left ventricle to right atrium (LV-RA) communication, is usually congenital. Acquired LV-RA communications are rare and only few case reports of successful trans-catheter closure have been published though none of them were on infants. We hereby report a rare case of LV to RA shunt acquired following surgical repair of Tetralogy of Fallot (TOF). The defect was successfully closed percutaneously with an Amplatzer duct occluder. This is the first reported case of device closure of an acquired Gerbode defect in an infant.

Objective: Heart rate variability (HRV) measures are altered in various cardiac and non-cardiac situations in children. The autonomic nervous system is assumed to have a role in the pathophysiology of atrial septal defect (ASD). In this study, we evaluated the autonomic system by measuring HRV in children with ASD., Methods: Twenty-eight patients with ASD and 32 healthy children (mean ages: 6.6±2.1 years and 6.4±2.2 years, respectively) were enrolled in the study. Twenty-four-hour ambulatory electrocardiographic recordings were obtained and the seven time-domain (SDNN, SDANN, rMSSD, SD, SDNN index, PNN50, and mean RR) and four frequency-domain (VLF, LF, HF, and LF/HF ratio) indices of HRV were analyzed., Results: A significant decrease in calculated HRV variables was observed in children with ASD as compared to controls. The HRV alteration was found in both time-domain and frequency-domain parameters., Conclusions: Our results indicate that HRV is decreased in children with ASD, which implies parasympathetic withdrawal and sympathetic predominance., (Copyright © 2013 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Background: The incidence of atrial septal defect (ASD) after percutaneous transvenous mitral commissurotomy (PTMC) ranges from 15.2% to 92% in small studies., Aim: To estimate the incidence of atrial septal defect (ASD) following PTMC and to determine the factors contributing to its development., Methods: We studied 209 patients with mitral stenosis (MS) undergoing PTMC. Transesophageal echocardiography (TEE) with color Doppler examination was performed to detect ASD., Results: TEE demonstrated ASD in 139 (66.5%) of 209 patients. The mean diameter of the interatrial septal defect detected by TEE was 4.47 ± 1.7 mm. The most common site of septal puncture was the inferior vena caval side of the interatrial septum followed by fossa ovalis. Color flow imaging across the defect showed left to right shunting in all the patients (100%). We examined the relationship of age, Wilkins score, left atrial volumes, the mitral valve orifice area, mitral valve gradient, and the degree of mitral and tricuspid regurgitation between the group that developed ASD and the group without ASD and found that none of these factors predicted the development of ASD. A residual ASD was seen in 11 patients (8.7%) at 6-month follow-up., Conclusion: Incidence of residual atrial septal defect immediately following PTMC by TEE color flow Doppler imaging is 66.5%. Surrogate markers of elevated left atrial pressures do not determine the development of atrial septal defect after PTMC. The majority of the defects close spontaneously and a residual defect is observed in 8.7% patients at 6 months., (© 2012, Wiley Periodicals, Inc.)

Protein regulation is the function of several pathways and enzyme systems in the human body. One of these pathways is the ubiquitin/SUMO pathway. The author has noted that almost all known syndromes of radial ray deficiency are related to this pathway. In this article, these syndromes are reviewed with special attention to their relationship with the ubiquitin/SUMO pathway. This opens a new insight into the pathogenesis of radial ray deficiency syndromes.