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.

Reliability of Echocardiographic Pulmonary Vascular Resistance to Screen for the New Definition of Precapillary Pulmonary Hypertension in Uncorrected Secundum Atrial Septal Defect

Background and Objective:The most feared complication of uncorrected secundum Atrial Septal Defect(ASD)is pulmonary arterial hypertension(PAH).Pulmonary vascular resistance(PVR)is crucial in detecting precapil-lary pulmonary hypertension(PH)to guide the need for PAH-specific therapy.There is a change in the cut-off value of PVR according to the recently updated PH guideline.How echocardiographic PVR(PVRecho)correlates to PVR by right heart catheterization(RHC)(PVRcath)according to the new guidelines has not been known.The aim of this study is to determine the reliability of PVRecho in detecting PAH in Uncorrected Ostium Secundum ASD based on the current updated guideline and to help screen the high PVR group.Methods:429 ostium secun-dum ASD in the COngenital HeARt Disease in Adult and Pulmonary Hypertension(COHARD-PH)registry was divided into three groups according to the PVR.PVRecho was calculated using Abbas’Formula and compared the its gold standard,the PVRcath.The correlation between the two methods was analyzed.The Bland-Altman plot was used to analyze the agreement between the two methods.Receiver operating characteristics(ROC)analysis was used to determine the PVRecho cut-off value for high PVR.Results:The majority of the population(63.5%)had high PVR.Female gender dominated the study population(84%).PVR_(echo) was significantly correlated with PVRcath(r=0.6225,p<0.0001).Bland-Altman plot among all groups and in subgroups analysis showed a wide range of agreement.PVRecho underestimated PVRcath 5.124 WU.In subgroup analysis,PVRecho overestimated PVRcath 0.35 WU in those with PVR<2 WU.In the second and third groups,PVR_(echo) underestimated PVRcath 0.52 and 10.77 WU,respectively.Conclusion:PVRecho is reliable in predicting high PVR in uncorrected secun-dum ASD.However,there is a wide range of agreement.PVR_(echo) cut-off value of>1.62 WU showed good dis-criminatory power in determining high PVR.

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.

Effects of atrial septal defects on the cardiac conduction system

The case report presented in this edition by Mu et al.The report presents a case of atrial septal defect(ASD)associated with electrocardiographic changes,noting that the crochetage sign resolved after Selective His Bundle Pacing(S-HBP)without requiring surgical closure.The mechanisms behind the appearance and resolution of the crochetage sign remain unclear.The authors observed the dis-appearance of the crochetage sign post-S-HBP,suggesting a possible correlation between these specific R waves and the cardiac conduction system.This editorial aims to explore various types of ASD and their relationship with the cardiac con-duction system,highlighting the diagnostic significance of the crochetage sign in ASD.

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.

“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.

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.

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.

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.

The Role of Transesophageal Echocardiography for Transcatheter Closure of Atrial Septal Defects with the Amplatzer Septal Occluder

Objective: To evaluate of the role of transesophageal echocardiography (TEE)in percutaneous closure of atrial septal defects (ASD) with the Amplatzer septal occluder. Methods:Sixty- two patients (10 to 55 years of age) were selected for percutaneous closure of ASD bytrans-esophageal echocardiography, which was also used to monitor the procedure, to select theappropriate size of the Amplatzer device, to verify its position, and to access the immediateresults of the procedure. During the follow-up, transthoracic echocardiography (TTE) or TEE was usedto evaluate the presence and magnitude of residual shunt (RS), device position, and right cardiacchamber diameters. Results: The mean ASD diameter by TTE ([19. 1 +- 5. 8] mm) was significantlysmaller (P< 0. 001) than the stretched diameter of the ASD (25. 1 +- 6. 4) mm. There are nosignificant differences between the TEE -measured value (23. 5+_6. 2) mm and the stretched diameterof the ASD (P > 0. 05). Due to proper patient selection all procedures were successful. There wasimmediate and complete closure in 61/62 patients, only one patients had trivial residual shunt.Follow- up was performed using TTE or TEE right after operation, 1 d, 1 month, 3 months, 6 monthsand yearly thereafter. Ail, patients remain asymptomatic without any clinical or technical problems.Conclusion: With the aid of TEE, percutaneous closure of ASD can be performed successfully, safely,and effectively.

Completed atrioventricular block induced by atrial septal defect occluder unfolding:A case report

BACKGROUND An atrial septal defect is a common condition and accounts for 25%of adult congenital heart diseases.Transcatheter occlusion is a widely used technique for the treatment of secondary aperture-type atrial septal defects(ASDs).CASE SUMMARY A 30-year-old female patient was diagnosed with ASD by transthoracic echocardiography(TTE)1 year ago.The electrocardiogram showed a heart rate of 88 beats per minute,normal sinus rhythm,and no change in the ST-T wave.After admission,TTE showed an atrial septal defect with a left-to-right shunt,aortic root short-axis section with an ASD diameter of 8 mm,a parasternal four-chamber section with an ASD diameter of 9 mm,and subxiphoid biatrial section with a diameter of 13 mm.Percutaneous occlusion was proposed.The intraoperative TTE scan showed that the atrial septal defect was oval in shape,was located near the root of the aorta,and had a maximum diameter of 13 mm.A 10-F sheath was placed in the right femoral vein,and a 0.035°hard guidewire was used to establish the transport track between the left pulmonary vein and the inferior vena cava.A shape-memory alloy atrial septal occluder with a waist diameter of 20 mm was placed successfully and located correctly.TTE showed that the double disk unfolded well and that the clamping of the atrial septum was smooth.Immediately after the disc was revealed,electrocardiograph monitoring showed that the ST interval of the inferior leads was prolonged,the P waves and QRS waves were separated,a junctional escape rhythm maintained the heart rate,and the blood pressure began to decrease.After removing the occluder,the elevation in the ST segment returned to normal immediately,and the sinus rhythm returned to average approximately 10 min later.After consulting the patient’s family,we finally decided to withdraw from the operation.CONCLUSION Compression of the small coronary artery,which provides an alternative blood supply to the atrioventricular nodule during the operation,leads to the emergence of a complete atrioventricular block.

Transcatheter to Close the Patent Duetus Arteriosus and Atrial Septal Defects in Children

Objective: To evaluate the therapeutic effect, safety and complications oftranscathetering Amplatzer device in the closure of patent ductus arteriosus (PDA) and atrial septaldefects (ASD) in children. Methods: Patients with PDA (n = 25) and ASD ( n = 16), confirmed byechocardiography, were treated by transcatheterization. Amplatzer occluder device was placed by thetranscatheterization with the image support of X-ray and transthoracic echocardiography ( TTE) . TheTTE, ECG and X-ray examination were engaged to evaluate the therapeutic results on the time pointsof 24 h , 1, 3 , 6, 12 months after the operation, and all these cases were engaged to the follow-upexamination. Results: The cardioangiographic diameter was 13.0-28.0 mm ([19.3+-4.9] mm) in ASD and2.0-7.7 mm ([3.9+-1.5] mm) in PDA . The diameter of the Amplatzer occluder selected were 13.0-30.0mm ( [20.6+-5.1] mm) in ASD and 4.0-12.0 mm ([6.6+-1.9] mm) in PDA , respectively. All the Amplatzeroccluders were placed successfully. There were no complications during and after the operation.Very small residual shunt was still found soon after the operation in 10 cases , and there were noresidual or recanalization after three months of the operation. The pulmonary artery pressure andheart size were significantly decreased in follow-up examination . Conclusion: Transcatheteringclosure with Amplatzer device is an effective, simple and safe technique in the treatment of ASD andPDA in children.

Radiofrequency ablation of atrial tachycardia in patients with repaired atrial septal defect

Objective:To evaluate the electrophysiological characteristics and radiofrequency catheter ablation of atrial tachycardia (AT) in patients with repaired atrial septal defects(ASD). Methods: In 76 consecutive patients with AT who underwent the electrophysiological study and radiofrequency catheter ablation (RFCA). 4 patients (one male and three female aged 35. 5±11. 5 years) had AT-related myocardial scar or incision. Earliest activation combined with entrainment mapping was adopted to determine a critical isthmus. Results: Re-entry related to the lateral atriotomy scar was inducible in 3 of 4 patients. With en-trainment mapping, the PPI-TCL difference was <30 ms when pacing at the inferior margins of the right lateral atriotomy scar. Among them, 2 patients had successful linear ablation between scar area to inferior vena cava, and 1 patient between scar areas to tricuspid annulus. Re-entry involving an ASD patch was demonstrated in 1 of 4 patients. PPI-TCL differences <30 ms were found when entraining tachycardia at sites near the septal patch. But linear ablation failed in terminating AT. There was no complication during procedure. No recurrence of incision-related AT was found during follow-up except for the failed patient. Conclusion: Under conventional electrophysiological mapping, adopting linear ablation from scar area to anatomic barrier, successful ablation also can be obtained in patients with IRAT related to myocardial scar or incision.

Superior Sinus Venosus Atrial Septal Defect: Overview of Surgical Options

Background: Superior sinus venosus atrial septal defect (SV-ASD) is an interatrial defect with partial anomalous pulmonary venous connection (PAPVC) draining into the right atrium. The principle for SV-ASD surgical repair involves redirecting the pulmonary venous blood back to the left atrium and closure of the atrial septal defect without compromising the adjoining structures. Methods: We reviewed English literature relating to this topic via a MEDLINE and Google scholar search using the following terms: surgery for sinus venosus atrial septal defect (SV-ASD), surgery for partial anomalous pulmonary venous connection (PAPVC) and complications of the surgery. Results: A total of 910 consecutive cohort patients with different surgical options between 1984 to 2012 were analyzed. The breakdown showed that 291(32.0%) had single-patch, 275(30.2%) had Warden’s procedure, 185(20.3%) had a double-patch and 159(17.5%) had use of autologous right atrial appendage for the anastomosis. The total follow-up was between 0.008-30 years. Sinus node dysfunction (SND) was the commonest complication occurring mostly in patients who had double-patch technique 16(8.6%) followed by 15(5.5%) patients with single-patch technique, 7(4.4%) patients in whom right atrial appendage was used and 5(1.8%) patients with Warden’s procedure. Venous obstruction occurred in 22(7.7%) patients with SP, 13(5.1%) patients with Warden’s Procedure and 5(2.7%) patients with double-patch. Conclusion: The two major complications, SND and venous obstruction, assumed a see-saw approach as surgical maneuvers avoiding venous obstruction encouraged SND and vice versa. However, adopting surgical options which avoided incision across the Cavoatrial junction attenuated all the complications.

Improvement of the Technique for Transcatheter Closure of Atrial Septal Defect in Children

Objectives To improve experience of procedure and success rate of interventional treatment of atrial septal defect （ASD） in children, applying the technique of controlling release of devices in the pulmonary vein （controlling two disc of device opening for subsequence） in children cases with ASD who can not be occluded by regularly interventional treatment. Methods Since 2000 year 182 child cases （male 70 and 112 female） underwent the procedure of controlling release of devices in the pulmonary vein. The patients＇ age was from 2 to 14 years old （average 3.77±1.55）. The body weight was from 9 to 48 Kg （average 21.53±10.63）. When the devices were placed on the right position with difficulty and failure in some cases with short and soft rims of the defect and large defect and the angle between the device and the interval atrial septal （IAS）, It could be helpful to put the device into the left upper pulmonary vein, and to make right atrium （proximal） disc opened before the left atrium （distal） disc naturally fall down. At the end the double disc of the device clamped and stood up at the right position of the IAS. After closure of ASD, patients were followed up regularly by echocardiography, X-ray and ECG in the 1,3,6,12 month and 3,5 years. Results The successful rate of device implantation in the improving group （98.4%） was obviously higher than that in the regularly group （68%）. The techniques improved in this group with the smaller age, the lighter weight, the larger defect and the larger device comparing with the regularly group. The velocity of the pulmonary vein before occlusion procedure was （0.54±0.15）m/s; after procedure was （0.56±0.16）m/s, P 〉 0.05,there were no significant difference. All cases couldn＇t found pulmonary congestion by follow up. Conclusions The method of controlling release of device in the pulmonary vein has been used more than 5 years in the occlusion of ASD with double disc device. It is feasible and safety. The aim of the improvement is for overcoming the problem with larger ASD and the rim deficiency in the interventional procedure in children with ASD. In summary： 1. The performance of the procedure must be careful; 2. To avoid complication when put the sheath into the pulmonary vein; 3. To avoid to put the left disc into pulmonary vein too long and pull the device too much; 4. When the procedure have to operate repeatedly, the device can not be released before the position satisfactory. The improvement of the technique is needed to carry on long-term follow-up in the clinical trials.

Device-related thrombosis on atrial septal defect occluder after simultaneous closure of left atrial appendage and atrial septal defect: a case report

An atrial septal defect (ASD) may cause right heart dysfunction, pulmonary hypertension and atrial fibrillation (AF), and atrial septal defect occlusion (ASDO) is the first choice for treating secundum defects when the morphology permits. ASD and AF frequently coexist, and the risk of AF and stroke persists after ASDO.[1] In recent years, left atrial appendage occlusion (LAAO) has been recognized as an effective treatment for stroke prevention in nonvalvular AF patients with a high risk of stroke, systemic embolism and bleeding who are unwilling to take oral anticoagulants or cannot tolerate them.

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.

Usefulness of myocardial performance index for assessing right ventricular function after percutaneous closure of atrial septal defect

Objective Assessment of right ventricular function in patients with atrial septal defect(ASD)is difficult.The Doppler myocardial performance index(MPI)may provide a method of assessing function in these patients.The purposes of this study were to evaluate the right ventricular function and its changes in patients with ASD after transcatheter closure of ASD.Methods MPI,defined as the sum of isovolumic relaxation time and isovolumic contraction time derived by ejection time,was measured from tricuspid inflow and right ventricular outflow;Doppler velocity profiles recorded during routine echocardiography.Twenty nine patients(13 men,16 women;mean age 25.28±12.69,range 6 to 57 years)were diagnosed to secundum ASD[the stretched diameters of ASD were from 9 To 36(24.91±7.98)mm],and had a successfully placed Amplatzer septal occluder(ASO)(the sizes of ASO were from 11 to 40 mm);there were 81 sex-matched,age-matched healthy people(control group 41men,40 women;mean age 29.02±14.22,range 4 to 45 years).MPI was measured again on 3 days and 1 month after closure of ASD.Change in the study group was assessed and compared to the control subjects with structurally normal hearts.A complete 2-dimensional and Doppler echocardiographic examination was performed in all study groups.Results 1)The isovolumic relaxation and isovolumic contraction times[respectively(77.59±14.39)ms vs(60.93±12.94)ms,P<0.0001;(28.28±10.88)ms vs(23.64±9.01)ms,P=0.027]were prolonged,and ejection time[(260.65±21.86)ms vs(271.85±21.92)ms,P=0.033]was shortened in patients with ASD compared with that in control subjects,resulting in a marked increase in the MPI(0.40±0.07 vs 0.31±0.05,P<0.0001)from normal values;2)by Pearson's correlations,the MPI had no correlation with heart rate and blood pressure in control subjects and patients with ASD,but it correlated positively with age in patients with ASD;3)by Pearson's correlations,the MPI correlated positively with the diameter of ASD and pulmonary artery pressure;4)after transcatheter closure of ASD,the MPI decreased markedly.Conclusions 1)MPI is a conceptually new,simple,and reproducible Doppler index in patients with ASD;2)MPI is free from the effect of age,heart rate and blood pressure;(3)MPI appears to be relatively dependent on changes in the diameter of ASD and pulmonary artery pressure;4)the right ventricular function was improved after transcatheter closure of ASD.

Home-made fenestrated amplatzer occluder for atrial septal defect and pulmonary arterial hypertension

We report the management of a patient with secundum atrial septal defect （ASD） and severe pulmonary hypertension. A 65-year-old male with recently diagnosed atrial septal defect was referred to our centre for decompensated right heart failure with rest and exercise induced dispnea and severe pulmonary hypertension. Right heart catheterization confirmed a mean pulmonary pressure of about 55 mmHg and a Qp/Qs of 2.7. An occlusion test with a compliant large balloon demonstrated partial fall of pulmonary arterial pressure. The implantation of a home-made fenestrated Amplatzer ASD Occluder （ASO） was planned in order to decrease left-to-right shunt and promote further decrease of pulmonary arterial pressure in the long-term. Thus, by means of mechanical intracardiac echocardiography study with a 9F 9 MHz Ultralce catheter （Boston Scientific Corp.）, we selected a 34 mm ASO for implantation. Four millimeter fenestration was made inflating a 4 mm non-compliant coronary balloon throughout the waist of the ASO, which was successfully implanted under intmcardiac echocardiography. After six months, a decrease of pulmonary arterial pressure to 24 mmHg and full compensated right heart failure was observed on transthoracic echocardiography and clinical examination. This case suggests that Wanscatheter closure with home-made fenestrated ASD in elderly patients with severe pulmonary hypertension is feasible.