Genetic Analysis of Variants of the MYH6 Gene Promoter in Congenital Atrial Septal Defects

Background:Atrial septal defect(ASD)is one of the common congenital heart diseases.The MYH6 gene has a critical role in cardiac development but the role of MYH6 promoter variants in patients with ASD has not been explored.Methods:In 613 subjects including 320 ASD patients,we investigated the MYH6 gene promoter variants and verified the effect on gene expression by using cellular functional experiments and bioinformatics analysis.Results:Eleven variants were identified in the MYH6 gene promoter,of which four variants were found only in ASD patients,and two variants(g.3434G>C and g.4524C>T)were identified for the first time.Cellular functional experiments indicated that all four variants reduced the transcriptional activity of the MYH6 gene promoter(p<0.05).Subsequent analysis through the JASPAR(A database of transcription factor binding profiles)suggests that these variants may alter transcription factor binding sites,which may in turn lead to changes in myocardin subunit expression and ASD formation.Conclusions:Our study for the first time focuses on variants in the promoter region of the MYH6 gene in Chinese patients with ASD and the discovered variants have functional significance.The study provides new insights in the role of the MYH6 gene promoter region to better understand the genetic basis of ASD formation and facilitates clinical diagnosis.

Percutaneous closure of secundum type atrial septal defects:More than 5-year follow-up

AIM: To investigate long-term efficacy of two different devices more than five years after percutaneous atrial septal defect(ASD) closure in adults.METHODS: All patients who underwent percutaneous closure of an ASD in the St. Antonius Hospital, Nieuwegein, The Netherlands, between February 1998 and December 2006 were included. Percutaneous closure took place under general anaesthesia and transesophageal echocardiographic moni toring. Transthoracic echocardiography(TTE) was performed 24 h post-procedure to visualize the device position and to look for residual shunting using color Doppler. All complications were registered. All patients were invited for an outpatient visit and contrast TTE more than 5-years after closure. Efficacy was based on the presence of a residual right-to-left shunt(RLS), graded as minimal, moderate or severe. The presence of a residual left-to-right shunt(LRS) was diagnosed using color Doppler, and was not graded. Descriptive statistics were used for patients' characteristics. Univariate analysis was used to identify predictors for residual shunting.RESULTS: In total, 104 patients(mean age 45.5 ± 17.1 years) underwent percutaneous ASD closure using an Amplatzer device(ASO) in 76 patients and a Cardioseal/Starflex device(CS/SF) in 28 patients. The mean follow-up was 6.4 ± 3.4 years. Device migration occurred in 4 patients of whom two cases occurred during the index hospitalization(1 ASO, 1 CS/SF). The other 2 cases of device migration occurred during the first 6 mo of follow-up(2 CS/SF). The recurrent thrombo-embolic event rate was similar in both groups: 0.4% per follow-up year. More than 12 mo post-ASD closure and latest follow-up, new-onset supraventricular tachyarrhythmia's occurred in 3.9% and 0% for the ASO and CS/SF group, respectively. The RLS rate at latest follow-up was 17.4%(minimal 10.9%, moderate 2.2%, severe 4.3%) and 45.5%(minimal 27.3%, moderate 18.2%, severe 0%) for the ASO- and CS/SF groups, respectively. There was no residual LRS in both groups.CONCLUSION: Percutaneous ASD closure has good long-term safety and efficacy profiles. The residual RLS rate seems to be high more than 5 years after closure, especially in the CS/SF. Residual LRS was not observed.

Clinical Value of Stereoscopic Three-dimensional Echocardiography in Assessment of Atrial Septal Defects: Feasibility and Efficiency

Stereoscopic three-dimensional echocardiography（S-3DE） is a novel displaying technol-ogy based on real-time 3-dimensional echocardiography （RT-3DE）. Our study was to evaluate the feasibility and efficiency of S-3DE in the diagnosis of atrial septal defect （ASD） and its use in the guidance for transcatheter ASD occlusion. Twelve patients with secundum ASD underwent RT-3DE examination and 9 of the 12 were subjected to transcatheter closure of ASD. Stereoscopic vision was generated with a high-performance volume renderer with red-green stereoscopic glasses. S-3DE was compared with standard RT-3D display for the assessment of the shape, size, and the surrounding tis-sues of ASD and for the guidance of ASD occlusion. The appearance rate of coronary sinus and the mean formation time of the IVC, SVC were compared. Our results showed that S-3DE could measure the diameter of ASD accurately and there was no significant difference in the measurements between S-3DE and standard 3D display （2.89±0.73 cm vs 2.85±0.72 cm, P〉0.05; r=0.96, P〈0.05）. The appearance of coronary sinus for S-3DE was higher as compared with the standard 3D display （93.3% vs 100%）. The mean time of the IVC, SVC for S-3DE monitor was slightly shorter than that of the standard 3D display （11.0±3.8 s vs 10.3±3.6 s, P〉0.05）. The mean completion time of interven-tional procedure was shortened with S-3DE display as compared with standard 3D display （17.3±3.1 min vs 23.0±3.9 min, P〈0.05）. Stereoscopic three-dimensional echocardiography could improve the visualization of three-dimensional echocardiography, facilitate the identification of the adjacent structures, decrease the time required for interventional manipulation. It may be a feasible, safe, and efficient tool for guiding transcatheter septal occlusion or the surgical interventions.

Missense mutations in CSX/NKX_(2.5)are associated with atrial septal defects

Objective ：To study the gene mutations of homeobox transcription factor （CSX/NKX2.5） associated with a Chinese family with secundum atrial septal defect （ASD）. Methods ：Polymerase chain reaction and DNA sequencing were used to check all the members in the family with ASD, and single strand conformation polymorphism analysis （SSCP） was used to check 126 normal control people for detecting the mutations of CSX/NKX2.5 gene. Results： Three mutations, G270A（Glu32Lys ）, G378A （Glu68Lys）andG390A （Glu72Lys）were identified in CSX/NKX2.5 gene of ASD patients. However, the other members in the family with ASD and the control did not have such gene mutations. Conclusion：These mutations of CSX/NKX2.5 gene, which were identified in a Chinese family, may be one of the secundum ASD etiologic causes .

Diagnostic chest X-ray in atrial septal defects

A 66-year-old woman with lower limb edema and exertional dyspnea presented to the emergency department.Posteroanterior chest X-ray revealed a Fleischner’s sign in both lungs(Figure 1A)suggesting a pulmonary hypertension.A subsequent transthoracic echocardiogram revealed a 24 mm diameter secundum atrial septal defect(ASD)(Figure 1B)with a dilated right heart and a mean pulmonary pressure of 68 mmHg.Fleischner’s sign refers to a prominent central pulmonary artery that can be commonly caused either by pulmonary hypertension or acute pulmonary embolism.This radiological finding is very rare in patients with secundum ASD unless the defect remains undiagnosed till the elderly.Chest X-ray remains fundamental in making diagnosis of cardiovascular disease.

TRANSCATHETER CLOSURE OF LARGE SECUNDUM ATRIAL SEPTAL DEFECTS WITH AMPLATZER~ SEPTAL OCCLUDER

Objective To evaluate the safety and feasibility of transcatheter closure of large secundum atrial septal defects(ASDs)with Amplatzer~ septal occluder(ASO).Methods A total of 26 patients(age 16 to 67 years,median 43 years;body weight 52 to 102 kg,median 67 kg)with large ASDs underwent an attempted transcatheter closure using ASO.Large ASD was defined as those with a balloon-stretched diameter of ≥30 mm.Another 81 patients with small-to-moderate ASD during same period who underwent closure served as controls.Results In patients with large ASDs,the ASD dimension means were(22.1±3.2)mm(range from 16 to 30 mm)and(23.8±2.6)mm(range from 18 to 31mm)assessed by transesophageal echocardiography(TEE)and transthoracic echocardiography(TTE),respectively.The mean balloon-stretched diameter of the ASD was(31.9±2.1)mm(range from 30 to 37mm).The size of device was(32.0±1.9)mm(range from 30 to 36mm).The transcatheter procedure was successful in all patients(100%).Seventeen deployments were performed using the conventional left atrium approach,and remaining 9 patients required the right upper pulmonary vein approach.Immediately after deployment,TEE revealed that complete closure rate was 73%.Procedure-related complications were recorded in 3 patients(12%),including device embolization in one patient,pericardial effusion in one patient,and large hematoma at puncture site in one patient.During follow-up,the complete closure rate increased to 88% at 24h after procedure and 100% at 6 months.The procedural success rates,immediate TEE results and TTE results at 24h and 6 months after procedure,were not significantly different between patients with large ASDs and those with small-to-moderate ASDs.Conclusion Transcatheter closure of large ASDs using ASO is technical feasible and relatively safe.Proper care selection and specific technique modification such as the right upper pulmonary vein approach is vital for the implantation success.

Impact of Atrial Septal Defect Closure on Mortality in Older Patients

Background:Atrial septal defect(ASD)is a common form of adult congenital heart disease that can lead to long-term adverse outcomes if left untreated.Early closure of ASD has been associated with excellent outcomes and lower complication rates.However,there is limited evidence regarding the prognosis of ASD closure in older adults.This study aims to evaluate the mortality rates in older ASD patients with and without closure.Methods:A retrospective cohort study was conducted on patients aged 40 years or older with ASD between 2001 and 2017.Patients were followed up to assess all-cause mortality.Univariable and multivariable analyses were performed to identify the predictors of mortality.A p-value of<0.05 was considered statistically significant.Results:The cohort consisted of 450 patients(mean age 56.6±10.4 years,77.3%female),with 66%aged between 40 and 60 years,and 34%over 60 years.Within the cohort,299 underwent ASD closure(201 with transcatheter and 98 with surgical closure).During the median follow-up duration of 7.9 years,51 patients died.The unadjusted cumulative 10-year rate of mortality was 3%in patients with ASD closure,and 28%in patients without ASD closure(log-rank p<0.001).Multivariable analysis revealed that age(hazard ratio[HR]1.04,95%confidence interval[CI]1.006–1.06,p=0.01),NYHA class(HR 2.75,95%CI 1.63–4.62,p<0.001),blood urea nitrogen(BUN)(HR 1.07,95%CI 1.03–1.12,p<0.001),right ventricular systolic pressure(RVSP)(HR 1.07,95%CI 1.003–1.04,p=0.01),and lack of ASD closure(HR 15.12,95%CI 5.63–40.59,p<0.001)were independently associated with mortality.Conclusion:ASD closure demonstrated favorable outcomes in older patients.Age,NYHA class,BUN,RVSP,and lack of ASD closure were identified as independent factors linked to mortality in this population.

Intelligent diagnosis of atrial septal defect in children using echocardiography with deep learning

Background Atrial septal defect(ASD)is one of the most common congenital heart diseases.The diagnosis of ASD via transthoracic echocardiography is subjective and time-consuming.Methods The objective of this study was to evaluate the feasibility and accuracy of automatic detection of ASD in children based on color Doppler echocardiographic static images using end-to-end convolutional neural networks.The proposed depthwise separable convolution model identifies ASDs with static color Doppler images in a standard view.Among the standard views,we selected two echocardiographic views,i.e.,the subcostal sagittal view of the atrium septum and the low parasternal four-chamber view.The developed ASD detection system was validated using a training set consisting of 396 echocardiographic images corresponding to 198 cases.Additionally,an independent test dataset of 112 images corresponding to 56 cases was used,including 101 cases with ASDs and 153 cases with normal hearts.Results The average area under the receiver operating characteristic curve,recall,precision,specificity,F1-score,and accuracy of the proposed ASD detection model were 91.99,80.00,82.22,87.50,79.57,and 83.04,respectively.Conclusions The proposed model can accurately and automatically identify ASD,providing a strong foundation for the intelligent diagnosis of congenital heart diseases.

Transcatheter versus surgical closure of secundum atrial septal defects:a systematic review and meta-analysis

Background Transcatheter closure and surgical closure of secundum atrial septal defects(SASDs)are common treatment methods.It is not clear which method has a more obvious technical advantage.Objective To summarise data from studies comparing transcatheter closure versus surgical closure of SASDs.Methods Databases including Pub Med,EMbase,Web of science,Cochrane Library,CBM,CNKI and Wanfang databases were searched to enroll randomized controlled trials and cohort study trials comparing the two treatment methods(transcatheter closure versus surgical closure).The literature was screened according to the inclusive and exclusive criteria by two reviewers independently.The quality of the trials was evaluated.The data were extracted and meta-analysis was performed using Rev Man 5.3 software.Results 29 original studies(14,941 patients)were included.All studies were non-random.Transcatheter closure was found to be superior to surgical closure in the following outcomes:all-cause mortality(OR 0.36,95%CI 0.21~0.59,P<0.0001),total complications(OR 0.37,95%CI 0.25~0.55,P<0.00001),and hospital stay length(WMD-3.77,95%CI-4.36~-3.18,P<0.00001).Residual shunts incidence and retreatment incidence were more common in the transcatheter closure group than those in the surgical closure group[(OR 3.55,95%CI 1.79~7.05,P=0.0003)and(OR 2.19,95%CI 1.31~3.65,P=0.003),respectively].Conclusions Both approaches are effective for closure SASDs.Transcatheter is associated with lower mortality,complications and hospital stay length,while surgical closure has incidence of residual shunting and retreatment.

Transcatheter closure of secundum atrial septal defects using Amplatzer device

To evaluate the feasibility and short term results of transcatheter closure of secundum atrial septal defects (TCSASDs) using Amplatzer occluder device A new self centering device, the Amplatzer atrial septal occluder, has recently been evaluated in an animal model with excellent results This paper reports our clinical research with this device Methods 30 patients underwent an attempt at TCSASDs at an average age of 35 6±15 9?y (range, 5-62?y) and average weight of 63 6±14 5?kg (range, 17-78?kg) using the Amplatzer atrial septal occluder The average ASD diameter measured by transesophageal echocardiography (TEE) was 23 6±4 0?mm (range, 16-31?mm), and the average atrial septal defects (ASD) balloon stretched diameter was 25 2±5 8?mm (range, 9-34?mm) All patients had right atrial and ventricular volume overload with a Qp/Qs of 3 4±1 2 (1 4-5 2) A F8 12 catheter was used for delivery of the device in all patients Results The device was placed correctly in all patients There was immediate and complete closure in 30 patients The average device waist size used to close the defect was 25 6±5 9?mm (range, 9-34?mm) There was no episode of device embolization or any other complication Follow up was performed using transthoracic echocardiography (TTE) 1 day, 1 month, 3 months, 6 months and 1 year after the procedure The first day after operation; there was complete closure of the ASD in all patients; there have been no episodes of endocarditis, thromboembolism, or wire fracture Conclusion TCSASDs using the Amplatzer occluder device was an efficient nonsurgical method which had a very high technical success rate and satisfactoy short term and follow up results

Reliability of transthoracic echocardiography in estimating the size of Amplatzer septal occluder and guiding percutaneous closure of atrial septal defects

Background In China, transthoracic echocardiography （TTE） is popularly used for pre-intervention examination for atrial septal defect （ASD） and for guiding ASD closure. However, the ability to determine ASD size and the safety and efficacy of l-rE for guiding ASD closure still has not been widely accepted. This study aimed to evaluate the efficacy and safety of l-rE used before, during and after transcatheter ASD closure with Amplatzer septal occluders （ASO）. Methods Sixty-eight subjects （15 men and 53 women; mean age （33.7±17.3） years） were enrolled. TTE was used to measure the diameters and guide transcatheter closure of ASD. The ASD was examined by long-axis view, basal short-axis view, apical four-chamber view and the subcostal view to observe position, diameter and relation with neighbouring structures. The largest diameter was selected as the reference diameter. Patients were divided into 3 groups according to the ASD reference diameter： 22 subjects with ASD diameter 4-14 mm （group A）; 21 subjects with ASD diameter 15-20 mm （group B）; and 25 subjects with ASD diameter 21-33 mm （group C）. Results ASD was occluded successfully in groups A and B. In group C, occlusion failed in 2 cases; 1 case remained with a 3-mm residual shunt sustained until 6-month follow-up. However, at 6-month follow-up, no case of thromboembolism, ASO dislocation or death occurred in the three groups. The diameter of ASD measured by l-rE could accurately predict the ASO size that could successfully occlude the ASD, especially in patients with ASD 〈20 mm. The ASD diameter measured by l-rE correlated well with ASO size （r= 0.925, P〈0.001 ; r=0.976, P〈0.001 ; r=0.929, P〈0.001 respectively）. Conclusions ASD diameter measured by l-rE can accurately estimate the size of the ASO needed for successful closure of ASD. The larger the ASD, the much larger the ASO needed. l-rE is a satisfactory guiding imaging tool for ASD closure.

“Treat-Repair-Treat”:Management of Left Main Coronary Compression by a Pulmonary Artery Aneurysm in a Patient with Atrial Septal Defect and Significant Pulmonary Hypertension

Left main coronary compression syndrome(LMCS)may complicate pulmonary artery aneurysms(PAA),usually developed in the context of pulmonary arterial hypertension(PAH).We report the case of a 51-year-old female patient with an atrial septal defect(unsuitable for device closure)complicated by a PAA generating a 90%left main stenosis.The significant PAH held us back from immediate surgery.After specific dual PAH-targeted therapy(sildenafil and bosentan),the atrial septal defect could be closed with a unidirectional valved patch;the PAAinduced LMCS was treated by reductive arterioplasty.The postoperative course was uneventful.Follow-up showed clinical improvement,but PAH treatment was still needed.After three months,coronary angiography showed only an insignificant residual left main stenosis,proving that reductive pulmonary arterioplasty was effective in treating LMCS.Any PAA requires further evaluation for LMCS,a dangerous but treatable complication.The“treat-repair-treat”strategy and shunt-closure with a unidirectional valved patch can both improve surgical prospects of LMCS with shunt-related PAH.

The Effect of Atrial Septal Defect Closure on Cardiac Volumetric Changes in Adults, Transcatheter Versus Surgical Closure, a Pilot Cardiac Magnetic Resonance Study

Background:Closure of an atrial septal defect(ASD)reduces right-side heart volumes by abolishing shunting with simultaneous improvement of the left ventricle(LV)filling and functions due to ventricular interdependence,thereby improving symptoms.Furthermore,studies conducted on atrial volume changes after ASD closure are limited.Cardiac magnetic resonance(CMR)is considered as the gold standard method for measuring cardiac volume and mass.Objective:We aimed to study the effect of transcatheter and surgical closure of secundum ASD on cardiac volumes and systolic functions as well as the fate of tricuspid regurgitation(TR),using CMR analysis.Methods:We prospectively enrolled 30 adult patients with isolated secundum ASD who were referred to ASD closure.CMR evaluation of cardiac chambers indexed volumes,systolic function,myocardial mass index,and tricuspid regurgitant fraction were done at before and 6 months after closure.Results:RV volumes decreased in both groups when compared to baseline(p-value 0.001),the device group had more reduction in volumes and more improvement in RV function after closure(p-value 0.001)when compared to the surgical arm.The changes in the RV mass index were insignificant between both groups(p-value 0.31).Functional TR improved to the same extent in both groups.Left ventricular end diastolic volume index(LVEDVI)and LV mass index increased sig-nificantly in both groups when compared to baseline in both groups but with no difference between groups p-value 0.01),left ventricular end systolic volume index(LVESVI)changes were insignificant.LV systolic function improved in patients who underwent device closure only(63.53±3.85 vs.67.13±4.34,p-value 0.01).There was a significant reduction in right atrial(RA)volumes and an insignificant decrease in left atrial(LA)volumes,with no difference between groups.Conclusion:Transcatheter and surgical secundum ASD closure resulted in volumetric changes in some cardiac chambers with better improvement in bi-ventricular systolic function in the transcatheter arm and no difference in the TR reduction between the two groups at 6 months follow-up by CMR.

Complications Related to Transcatheter Occlusion of Atrial Septal Defect with Amplatzer Septal Occluder in Children

Objectives To investigate the causes, theraputic and preventive methods of com- plications associated with transcatheter occlusion of at- rial septal defect ( ASD) using the Amplatzer septal oc- cluder (ASO) in children. Methods 289 cases un- derwent transcatheter closure of ASD with ASO. Com- plications occurred in 9 cases. The complications in procedure included systemic circulatory systemic air embolism in 2, pulmonary air embolism in 1, pericar- dial tamponade in 1, ASO malposition requiring emer- gency surgical removal in 1, transient atrial extrasysto- les in 1 and sizing balloon rupture in 1 case. 2 cases with postoperative complications were found in the fol- low-up studies. These included perforation of mitral valve and ASO partially dislodged. In this study, 5 children with intraoperative complication received e- mergency therapy including surgical intervention, and others needed only follow-up, as the complications were transient or asymptomatic. Results There were no children death in this study. The 5 cases who re- ceived treatment were completely healing, and the oth- ers with intra - procedure complications were also had no sequela existed. Cases with mitral valve and ASO partially dislodged were still in follow-up studies, as the 2 patients having no symptoms. Conclutions Air embolism were occurred easily in atrial septal defect cases who received ASO therapy. Complications mainly caused by inappropriate operative procedure and some complications needed emergency treatments. Follow-up studies were important to cases with transcatheter oc- clusion therapy.

Delayed cardiac tamponade after simultaneous transcatheter atrial septal defect closure and left atrial appendage closure device implantation: a particular case report

Percutaneous left atrial appendage(LAA)occlusion evolved as an alternative treatment to the patients who are contraindicated or cannot tolerate oral anticoagulants with nonvalvular atrial fibrillation(AF)at risk of stroke or systemic embolism.[1]Abnormal hemodynamic changes in elder atrial septal defect(ASD)patients cause remodeling of the left atrium,which eventually leads to right heart failure.[2]As the ASDs elderly are associated with a higher incidence of AF,simultaneous transcatheter ASD and LAA closure has become a new effective therapeutic strategy.However,only a limited number of articles involving cardiac tamponade complications have been published in the literature.What’s more,previous studies involving early hemodynamically irrelevant pericardial effusion after the procedure attribute to multiple repositioning attempts of LAA occluder or delivery sheath injured the atrial wall.

Evaluation of Atrial Septal Defect Using Real-time Three-dimensional Echocardiography:Comparison with Surgical Findings

The present study evaluated the application of three dimensional echocardigraphy （3DE） in the diagnosis of atrial septal defect （ASD） and the measurement of its size by 3DE and compared the size with surgical findings. Two-dimensional and real-time three dimensional echocardiography （RT3DE） was performed in 26 patients with atrial septal defect, and the echocardiographic data were compared with the surgical findings. Significant correlation was found between defect diameter by RT3DE and that measured during surgery （r=0.77, P〈0.001）. The defect area changed significantly during cardiac cycle. Percentage change in defect size during cardiac cycle ranged from 6%-70%. Our study showed that the size and morphology of atrial septal defect obtained with RT3DE correlate well with surgical findings. Therefore, RT3DE is a feasible and accurate non-invasive imaging tool for assessment of atrial septal size and dynamic changes.

Dynamic Characteristic Mechanism of Atrial Septal Defect Using Real-Time Three-Dimensional Echocardiography and Evaluation of Right Ventricular Functions

The dynamic characteristics of the area of the atrial septal defect（ASD） were evaluated using the technique of real-time three-dimensional echocardiography（RT 3DE）, the potential factors responsible for the dynamic characteristics of the area of ASD were observed, and the overall and local volume and functions of the patients with ASD were measured. RT 3DE was performed on the 27 normal controls and 28 patients with ASD. Based on the three-dimensional data workstations, the area of ASD was measured at P wave vertex, R wave vertex, T wave starting point, and T wave terminal point and in the T-P section. The right atrial volume in the same time phase of the cardiac cycle and the motion displacement distance of the tricuspid annulus in the corresponding period were measured. The measured value of the area of ASD was analyzed. The changes in the right atrial volume and the motion displacement distance of the tricuspid annulus in the normal control group and the ASD group were compared. The right ventricular ejection fractions in the normal control group and the ASD group were compared using the RT 3DE long-axis eight-plane（LA 8-plane） method. Real-time three-dimensional volume imaging was performed in the normal control group and ASD group（n=30）. The right ventricular inflow tract, outflow tract, cardiac apex muscular trabecula dilatation, end-systolic volume, overall dilatation, end-systolic volume, and appropriate local and overall ejection fractions in both two groups were measured with the four-dimensional right ventricular quantitative analysis method（4D RVQ） and compared. The overall right ventricular volume and the ejection fraction measured by the LA 8-plane method and 4D RVQ were subjected to a related analysis. Dynamic changes occurred to the area of ASD in the cardiac cycle. The rules for dynamic changes in the area of ASD and the rules for changes in the right atrial volume in the cardiac cycle were consistent. The maximum value of the changes in the right atrial volume occurred in the end-systolic period when the peak of the curve appeared. The minimum value of the changes occurred in the end-systolic period and was located at the lowest point of the volume variation curve. The area variation curve for ASD and the motion variation curve for the tricuspid annulus in the cardiac cycle were the same. The displacement of the tricuspid annulus exhibited directionality. The measured values of the area of ASD at P wave vertex, R wave vertex, T wave starting point, T wave terminal point and in the T-P section were properly correlated with the right atrial volume（P〈0.001）. The area of ASD and the motion displacement distance of the tricuspid annulus were negatively correlated（P〈0.05）. The right atrial volumes in the ASD group in the cardiac cycle in various time phases increased significantly as compared with those in the normal control group（P=0.0001）. The motion displacement distance of the tricuspid annulus decreased significantly in the ASD group as compared with that in the normal control group（P=0.043）. The right ventricular ejection fraction in the ASD group was lower than that in the normal control group（P=0.032）. The ejection fraction of the cardiac apex trabecula of the ASD patients was significantly lower than the ejection fractions of the right ventricular outflow tract and inflow tract and overall ejection fraction. The difference was statistically significant（P=0.005）. The right ventricular local and overall dilatation and end-systolic volumes in the ASD group increased significantly as compared with those in the normal control group（P=0.031）. The a RVEF and the overall ejection fraction decreased in the ASD group as compared with those in the normal control group（P=0.0005）. The dynamic changes in the area of ASD and the motion curves for the right atrial volume and tricuspid annulus have the same dynamic characteristics. RT 3DE can be used to accurately evaluate the local and overall volume and functions of the right ventricle. The local and overall volume loads of the right ventricle in the ASD patients increase significantly as compared with those of the normal people. The right ventricular cardiac apex and the overall systolic function decrease.

Cryoballoon pulmonary vein isolation and left atrial appendage occlusion prior to atrial septal defect closure: A case report

BACKGROUND In patients who suffer from both atrial fibrillation(AF)and atrial septal defect(ASD),cryoballoon pulmonary vein isolation(PVI),sequential left atrial appendage(LAA)occlusion and ASD closure could be a strategy for effective prevention of stroke and right heart failure.CASE SUMMARY A 65-year-old man was admitted to our institution due to recurrent episodes of palpitations and shortness of breath for 2 years,which had been worsening over the last 48 h.He had a history of AF,ASD,coronary heart disease with stent implantation and diabetes.Physical and laboratory examinations showed no abnormalities.The score of CHA2DS2VASc was 3,and HAS-BLED was 1.Echocardiography revealed a 25-mm secundum ASD.Pulmonary vein(PV)and LAA anatomy were assessed by cardiac computed tomography.PV mapping with 10-pole Lasso catheter was performed following ablation of all four PVs with complete PVI.Following the cryoballoon PVI,the patient underwent LAA occlusion under transesophageal echocardiographic monitoring.Lastly,a 34-mm JIYI ASD occlude device was implanted.A follow-up transesophageal echocardiography at 3 mo showed proper position of both devices and neither thrombi nor leakage was found.CONCLUSION Sequential cryoballoon PVI and LAA occlusion prior to ASD closure can be performed safely in AF patients with ASD.

Misdiagnosis of unroofed coronary sinus syndrome as an ostium primum atrial septal defect by echocardiography:A case report

BACKGROUND Unroofed coronary sinus syndrome(UCSS)is a rare congenital heart disease,which has variable morphologic features and is strongly associated with persistent left superior vena cava(PLSVC).However,it is often difficult to visualize the left-to-right shunt pathway through the CS by transthoracic echocardiography(TTE).CASE SUMMARY A 37-year-old female was admitted to the hepatological surgery department of a hospital with complaint of subxiphoid pain that had started 1 wk prior.Physical examination revealed a grade 3/6 systolic murmur at the left margin of the sternum,between the 2nd and 3rd intercostal cartilage.The patient underwent echocardiography and was diagnosed with ostium primum atrial septal defect(ASD);thus,she was subsequently transferred to the cardiovascular surgery department.A second TTE evaluation before surgery showed type IV UCSS with secundum ASD.Right-heart contrast echocardiography(RHCE)showed that the right atrium and right ventricle were immediately filled with microbubbles,but no microbubble was observed in the CS.Meanwhile,negative filling was observed at the right atrium orifice of the CS and right atrium side of the secundum atrial septal.RHCE identified UCSS combined with secundum ASD but without PLSVC in this patient.CONCLUSION This rare case of UCSS highlights the value of TTE combined with RHCE in confirming UCSS with ASD or PLSVC.

Efficacy and Safety of the Atrial Septal Defect Closure for Patients with Absent or Malaligned Aortic Rim Using a Figulla Flex II Device Flared and Straddling Behind the Aorta

Background:Although transcatheter closure of atrial septal defect(ASD)is safe and effective for patients with sufficient rim,ASD patients with absent and/or malaligned aortic and/or superior rim have higher risks of device embolization and cardiac erosion.We have treated such high-risk patients using a Figulla Flex II(FFII)device shaped flared and straddling behind the aorta because this method would avoid such serious complications.However,its long-term efficacy and safety remain unclear.Therefore,the midterm efficacy and safety of this method were studied.Methods:We retrospectively evaluated the outcome of 47 consecutive patients with such rim(age 6–73 years,weight 17–75 kg,31 females)treated with this method at our hospital between February 2016 and September 2019.To make the flared and straddling shape,we selected a FFII 4–6 mm larger than the balloon sizing diameter by stop-flow technique.We also studied the device shape,the disc pressure to the Valsalva wall and their changes over 6 months by transesophageal echocardiography.Results:All procedures were successful,and leakage disappeared within 1 year.During a mean follow up of 37±12 months,complications included a transient sinus node dysfunction and one I°atrioventricular block within 3 months.Whole device shape changed from bulky to thin:the device waist and thickness significantly decreased by around 1.5 mm and 3.5 mm,respectively(p<0.05),but the two discs remained flared and straddling behind the aorta over 6 months;therefore,the disc edges seldom pressed the Valsalva wall perpendicularly,even though the inner plane of either disc often slightly pressed the wall.Conclusions:ASD closure using a FFII shaped flared and straddling behind the aorta is probably effective and safe for patients with absent and/or malaligned aortic and/or superior rim although requiring care for conduction disorders.