Diagnosis Of Atrial Septal Defect Research Articles

Transcatheter Closure of Atrial Septal Defect with Amplatzer Device in Adolescence and Adults: Short Term Results: MCVTC Experience

Introduction: Atrial septal defect (ASD) is one of the common congenital heart diseases accounting for 8% to 10% of total congenital heart disease at birth and one of the most common diseases in adulthood. Ostium Secundum (OS) ASD has been successfully closed surgically for over several decades. But even though the results are better, it is associated with discomfort, morbidity, and a thoracotomy scar. Transcatheter closure in these patients has advanced rapidly in recent years and represents a growing field in invasive cardiology. Methods: This prospective study was conducted at Manmohan cardiothoracic vascular and transplant center, Maharajgung, Kathmandu, Nepal. Fifty-two (52) patients with a diagnosis of OS ASD assessed for device closure. Patients underwent transthoracic and trans oesophageal echocardiography for the defect location, size, number, and associated anomalies. All patients with OS ASD fulfilling the inclusion criteria enrolled for device closure. The procedure conducted under fluoroscopy and transesophageal guidance. The cases were followed up at 3 and 6 months, reassessed clinically and by echocardiography for any complications. Results: The patients with ASD who enrolled in this study belonged to the age group of 14 to 63 years. The device closure attempted in 46 patients, and the remaining six patients excluded from the procedure due to unsuitable anatomy after GA guided TEE done. Among 46 patients, 44 (95%) had successful device closure. The mean age of the patient was 35.7+ 10 and 33 (71.7%) female, with 13 (28.3%) males. Conclusions: The transcatheter device closure of ASD in adolescents and adults has a high success rate with fewer complications and found to be safely documented during immediate and short term follow up. However, long terms follow up is required to conclude it.

Mutations in the tail domain of MYH3 contributes to atrial septal defect.

Atrial septal defect (ASD) is one of the most common congenital heart defects diagnosed in children. Sarcomeric genes has been attributed to ASD and knockdown of MYH3 functionally homologues gene in chick models indicated abnormal atrial septal development. Here, we report for the first time, a case-control study investigating the role of MYH3 among non-syndromic ASD patients in contributing to septal development. Four amplicons which will amplifies the 40 kb MYH3 were designed and amplified using long range-PCR. The amplicons were then sequenced using indexed paired-end libraries on the MiSeq platform. The STREGA guidelines were applied for planning and reporting. The non-synonymous c. 3574G>A (p.Ala1192Thr) [p = 0.001, OR = 2.30 (1.36–3.87)] located within the tail domain indicated a highly conserved protein region. The mutant model of c. 3574G>A (p.Ala1192Thr) showed high root mean square deviation (RMSD) values compared to the wild model. To our knowledge, this is the first study to provide compelling evidence on the pathogenesis of MYH3 variants towards ASD hence, suggesting the crucial role of non-synonymous variants in the tail domain of MYH3 towards atrial septal development. It is hoped that this gene can be used as panel for diagnosis of ASD in future.

Evaluation of Clinical Course in Children and Adolescents with Atrial Septal Defects

Background Atrial septal defects (ASDs) are the most common third congenital heart defects. This study aimed to evaluate the clinical course of ASDs and the relationship between its complications, location closure and size. Materials and Methods: This cross-sectional study was conducted in the cardiac center of the pediatric ward in Zahedan, Iran. The study was carried out on 529 children with ASD between 2003 and 2018. The ASD children underwent echocardiography and complete examination such as physical exams, ECG and chest X-ray at every visit during follow-up. A diagnosis of ASDs was confirmed by a transthoracic echocardiography. A unique cardiologist applied transthoracic echocardiography to get information about size, location, and the number of the defects as well as hemodynamic information such as pulmonary artery pressure and any associated lesions. The data were analyzed using SPSS software version 20.0. Results: From 529 ASD children, 278 (52.5%) were girls. Most were medium (46.1%). 44.2% were closed by surgery; about 90.9% were secundum. 133 closed spontaneously and 14.6% by device. ASDs size had significant association with closure, location, and complication (P<0.001). The sinus venosus occurred in 29 patients, of which 62.07% and 37.93% were medium and large, respectively. PH was observed in nine children, 88.89% were large. ASD closure had significant association with location, and complication (P<0.001). From secundums, surgery and occluder devices closed 40.75% and 15.80, respectively. From those closed by surgery, 8.12% had residuals, 10.26% were partial anomalous pulmonary venous connection (PAPVC) as comorbidities, and 3.42% had pulmonary hypertension. Conclusion: From the study concluded ASDs size had significant association with closure, location, and complication and ASDs closure had significant association with location and complication.

Increased Risk of Perioperative Ischemic Stroke in Patients Who Undergo Noncardiac Surgery with Preexisting Atrial Septal Defect or Patent Foramen Ovale

To evaluate whether a preoperative diagnosis of atrial septal defect (ASD) or patent foramen ovale (PFO) is associated with perioperative stroke in noncardiac surgery and their outcomes. Retrospective cohort analysis. United States hospitals. Adults patients (≥18 years old) who underwent major noncardiac surgery from 2010 to 2015 were identified using the Healthcare Cost and Utilization Project's National Readmission Database. Preoperative diagnosis of ASD or patent foramen ovale. Among the 19,659,161 hospitalizations for major noncardiac surgery analyzed, 12,248 (0.06%) had a preoperative diagnosis of ASD/PFO. Perioperative ischemic stroke occurred in 723 (5.9%) of patients with ASD/PFO and373,291 (0.02%) of those without ASD/PFO (adjusted odds ratio [aOR], 16.7; 95% confidence interval [CI]: 13.9-20.0). Amongst the different types of noncardiac surgeries, obstetric, endocrine, and skin and burn surgery were associated with higher risk of stroke in patients with pre-existing ASD/PFO. Moreover, patients with ASD/PFO also had an increased in-hospital mortality (aOR, 4.6, 95% CI: 3.6-6.0), 30-day readmission (aOR, 1.2, 95% CI: 1.04-1.38), and 30-day stroke (aOR, 7.2, 95% CI: 3.1-16.6). After adjusting for atrial fibrillation, ischemic stroke remained significantly high in the ASD/PFO group (aOR: 23.7, 95%CI 19.4-28.9), as well as in-hospital mortality (aOR: 5.6, 95% CI 4.1-7.7), 30-day readmission (aOR: 1.19, 95%CI 1.0-1.4), and 30-day stroke (aOR: 9.3, 95% CI 3.7-23.6). Among adult patients undergoing major noncardiac surgery, pre-existing ASD/PFO is associated with increased risk of perioperative ischemic stroke, in-hospital mortality, 30-day stroke, and 30-day readmission after surgery.

Pulmonary hypertension after shunt closure in patients with simple congenital heart defects

BackgroundPatients with simple shunt lesions, such as atrial septal defect (ASD), ventricular septal defect (VSD) and persistent arterial duct (PDA) remain at risk of developing pulmonary hypertension (PH) even after correction of their cardiac defect. We aimed to assess the contemporary prevalence of PH in a well characterized nationwide group of patients based on the German National Register for Congenital Heart Defects. Methods and resultsWe included all patients >16 years of age with an isolated diagnosis of ASD, VSD or PDA. Only patients with previous surgical or interventional closure of the defect were included. Patients with genetic syndromes were excluded. Out of 49,597 CHD patients in the register we identified 825 patients with closed, isolated simple defects (52% ASD, 41% VSD, 7% PDA). Of these, 25 (3%) developed PH after a median follow-up of 16 years from defect closure. The risk of PH increased significantly with age at follow-up (p < 0.0001) and age at repair (p < 0.0001) on logistic regression analysis Patients with PH were significantly more likely to be symptomatic (59% vs. 9% in NYHA class ≥2, p < 0.0001) and had significantly higher mortality (hazard ratio 13.4, p < 0.0001) compared to the remaining patients. ConclusionsBased on data from the German National Register CHD Register we report a PH prevalence of 3.0% in patients with corrected, simple lesions. Patients with PH were more symptomatic and had significantly increased mortality risk. Life-long surveillance and low threshold for workup is recommended to ascertain diagnosis of PH, which has important prognostic and clinical implications.

Combined lung transplantation and percutaneous septal defect closure for end-stage atrial septal defect associated pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare but severe complication of unrepaired atrial septal defect (ASD). In patients with ASD, PAH and right ventricle failure, heart-lung transplantation (HLT) is currently viewed as the best ultimate procedure but is limited by the low number of grafts. We aim to report our preliminary experience of double lung transplantation (DLT) followed by percutaneous ostium secundum ASD closure in patients with end-stage ASD associated PAH. We report 6 patients with end-stage ASD associated PAH treated with DLT followed by percutaneous ASD closure. Median ages at ASD diagnosis and PAH diagnosis were respectively 22 y.o (min 0, max 37) and 16 (min 2 max 40). Median mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance before DLT were 78 mmHg (min 49, max108) and 14.0WU.m 2 (min 12.5, max 17.4) respectively. Median age at DLT was 29 y.o (min 20, max 44). Severe primary graft dysfunction occurred in 3 (50%) patients and was successfully managed medically. Post operative extracorporeal membrane oxygenation was required in 5 (83%) patients. Median ventilation time was 12 days (min 2, max 20). Median delay between DLT and percutaneous ASD closure occlusion was 4.4 months (min 3.9 max 18.7). Percutaneous closure was achieved using Amplatzer septal occluder, size running from n°16 to 40. mPAP was within normal range in all patient before ASD closure. All patient had improvement of their respiratory functional tests. All patients had improvement of right ventricular function ( Fig. 1 ) and none of them developed any chronic lung allograft dysfunction. Median follow up after lung transplant was 397 months (min: 265; max: 562). One patient died of pulmonary infection complication at 14.5 months after lung transplant. DLT followed by percutaneous ASD closure is an efficient therapeutic approach in patients with end-stage ASD associated PAH that may offer an alternative option to HLT.

Pregnancy outcome in women with atrial septal defect: associated with in vitro fertilisation and pre-eclampsia

ObjectiveTo investigate in vitro fertilisation (IVF) in women with atrial septal defect (ASD), and to examine the maternal characteristics and outcome of pregnancy, as well as the fetal outcome of...

Simple Radiography can Rescue from Dilemma

24 year old young lady came to us with a diagnosis of atrial septal defect (ASD) secundum who lost her follow up since her 4 years of age. Later her history, physical findings and investigations change the diagnosis into supracardiac total anomalous pulmonary venous drainage. Typical ‘snowman sign’ in chest x-ray and transthoracic echo helped to review the case. Proper history taking and careful evaluation by investigation is needful for each individual.&#x0D; Cardiovasc. j. 2019; 12(1): 75-78

P469440-year trends in incidence of simple congenital heart disease: a nationwide study

Abstract Background The methods for diagnosing congenital heart diseases (CHD) have changed radically over the last two decades. Since 2004, foetuses have been screened nationwide for congenital diseases in Denmark. In addition, the use of echocardiography has increased considerably during the last decades, facilitating the diagnosis of asymptomatic CHD. Despite the increased diagnostic possibilities, novel research regarding the incidence of simple CHD is lacking. Purpose To examine temporal trends in the incidence of simple CHD in the Danish population. Methods All Danish residents diagnosed with a ventricular septal defect (VSD), atrial septal defect (ASD) or patent ductus arteriosus (PDA) between 1 January 1977 and 31 December 2016 were identified by linking nationwide registers. Patients with simple CHD were defined as individuals diagnosed with an isolated VSD, ASD or PDA. Excluded was patients who had genetic disorders, other CHDs or developed a clinically significant morbidity such as myocardial infarction and chronic kidney disease before the age of 15 years. Results We included 10,464 patients diagnosed with simple CHD of whom 4,766 (45.5%) were men. During the study period, 4,317 (41.3%) were diagnosed with VSD, 4,148 (39.6%), with ASD and 1,999 (19.1%) with PDA. Crude incidence rates (IR) of ASD (Figure 1, panel A) remained unchanged between 1977–2004, after which ASD rates increased markedly until reaching a plateau between 2013–2016. Between 1997–2016, the median age at diagnosis of ASD increased substantially (Figure 1, panel B) (median age in 1997 = 19 years (IQR 11–45); in 2016 = 40 years (IQR 1–56); in 1977–1996: 18 years (IQR 5–44); in 1997–2016: 31 years (IQR 1–52)). VSD rates did not change between 1977–1991, increased until 1994 (IR in 1991 = 14.46 per 1 mil person years (PY); in 1994 = 26.06 per 1 mil PY) and subsequently slowly decreased until 2016 (IR in 2016 = 13.99 per 1 mil PY). The median age at diagnosis of VSD was consistent throughout the study period and was overall 1 years (IQR 0–17). The rates of PDA were consistent during the study period, and the overall median age at diagnosis of PDA was 1 months (IQR 1–40). Figure 1 Conclusion In this nationwide study of simple CHD, the incidence rates and age at time of diagnosis of VSD and PDA remained stable during the 40-year period. Contrastingly, the incidence rates and age at time of diagnosis of ASD increased markedly from 2004 and onwards. Acknowledgement/Funding The Danish Heart Foundation

Effect of Atrial Septal Defect in Adults on Work Participation (from a Nation Wide Register-Based Follow-Up Study Regarding Work Participation and Use of Permanent Social Security Benefits)

Low work participation is well known in patients with chronic disease but has not been described in patients with atrial septal defect (ASD). In this nation-wide cohort study, we report the first long-term follow-up of use of permanent social security benefits and work participation in adults with ASD. All Danes born before 1994 and diagnosed with ASD from 1959 to 2013 (n = 2,277) were identified from the Danish medical registries. We used Cox proportional hazards regression to compare the risk of receiving permanent social security benefits in the ASD patients compared with an age- and gender-matched general population cohort. Using the DREAM database, we calculated work participation score and proportion of patients working or not working at the age of 30 years. Median follow-up from ASD diagnosis was 23.4 years (range 0.2 to 59.3). ASD patients had a higher risk of receiving permanent social security benefits (hazard ratio 2.3 [95% confidence interval 2.1 to 2.6]) compared with the comparison cohort with 24% of the ASD patients receiving permanent social security benefits at the end of follow-up compared with 12% of the comparison cohort. At the age of 30 years, the proportion not working was 28% in the ASD cohort and 18% in the comparison cohort. In patients with ASD, 23% of those without a job had a psychiatric diagnosis. In conclusion, the risk of receiving permanent social security benefits was twice as high in patients with ASD and the work participation score was reduced compared with the background population.

Combined lung transplantation and percutaneous septal defect closure for end-stage atrial septal defect associated pulmonary arterial hypertension

IntroductionPulmonary arterial hypertension (PAH) is a rare but severe complication of unrepaired atrial septal defect (ASD). In patients with ASD, PAH and right ventricle failure, heart-lung transplantation (HLT) is currently viewed as the best ultimate procedure but is limited by the low number of grafts. We aim to report our preliminary experience of double lung transplantation (DLT) followed by percutaneous ostium secundum ASD closure in patients with end-stage ASD associated PAH. MethodsWe report 6 patients with end-stage ASD associated PAH treated with DLT followed by percutaneous ASD closure. ResultsMedian ages at ASD diagnosis and PAH diagnosis were respectively 22 (min:0, max: 37) and 16 (min: 2 max: 40) year-old. Median mean pulmonary artery pressure and pulmonary vascular resistance before DLT were 78mmHg (min: 49, max: 108) and 14.0 WU.m2 (min: 12.5, max: 17.4) respectively.Median age at DLT was 29 (min 20, max 44) year-old. Severe primary graft dysfunction occurred in 3 (50%) patients and was successfully managed medically. Post operative extracorporeal membrane oxygenation was required in 5 (83%) patients. Median ventilation time was 12 days (min: 2, max 20). Transient tracheotomy was required in 2 patients. Median time of stay in intensive care unit was 15 days (min:7, max: 26).Median delay between DLT and percutaneous ASD closure occlusion delay was 4.4 months (min: 3.9 max: 18.7). Percutaneous closure was achieved using Amplatzer septal occluder in all patients (size running from n° 16 to 40). Mean pulmonary arterial pressure was within normal range in all patient before ASD closure. All patient had improvement of their respiratory functional tests. All patients had improvement of there right ventricular function (Fig. 1) and none of them developed any chronic lung allograft dysfunction.Median follow-up after lung transplant was 397 months (min: 265; max: 562). One patient died of pulmonary infection complication at 14.5 months after lung transplant. ConclusionDLT followed by percutaneous ASD closure is an efficient therapeutic approach in patients with end-stage ASD associated PAH that may offer an alternative option to HLT.

Atrial Septal Defect and the Risk of Ischemic Stroke in the Perioperative Period of Noncardiac Surgery

Stroke is a serious complication of noncardiac surgery. Congenital defects of the interatrial septum may be a potent risk factor for perioperative stroke. The aim of the present study was to determine the association between atrial septal defect (ASD) or patent foramen ovale (PFO) and in-hospital perioperative ischemic stroke after non-cardiac surgery in a large nationwide cohort of patients hospitalized in the United States. Patients undergoing noncardiac surgery between 2004 and 2014 were identified using the Healthcare Cost and Utilization Project's National Inpatient Sample. Patients without an in-hospital echocardiogram were excluded. The presence of an ostium secundum-type ASD or PFO was identified by ICD-9 diagnosis code 745.5. The primary study outcome was perioperative acute ischemic stroke. Between 2004 and 2014, there were 639,985 admissions for noncardiac surgery with an in-hospital echocardiogram. An ASD or PFO was documented in 9,041 (1.4%) hospitalizations. Perioperative ischemic stroke occurred more frequently in patients with an ASD or PFO compared with those without an ASD or PFO (35.1% vs 6.0%, p <0.001). The association between ASD or PFO and ischemic stroke persisted after adjustment for demographics and clinical covariates (adjusted odds ratio 6.30, 95% confidence interval, 5.59 to 7.10) and in all non-cardiac surgery subtypes. In conclusion, in a large, nationwide analysis of patients undergoing noncardiac surgery, a diagnosis of ASD or PFO was associated with an increased risk of acute ischemic stroke overall and in all surgical subtypes. Additional measures are necessary to mitigate stroke risk in patients with septal defects who are planned for non-cardiac surgery.

Sinus venosus versus ostium secundum atrial septal defects: Their diagnosis and surgical outcome

Background: Diagnosis of sinus venosus atrial septal defects (ASDs) poses clinical difficulties than that of ostium secundum ASDs which may delay its diagnosis and treatment. Surgical repair of sinus venosus ASDs is more intricate in correlation with the repair of ostium secundum ASDs and conveys the risk of stenosis of the superior vena cava (SVC) or the rerouted pulmonary veins (PVs) and sinus node dysfunction (SND). Objectives: To compare diagnostic modalities and surgical outcome of sinus venosus ASDs to that of ostium secundum ASDs. Patients and Methods: Out of the total 72 patients in the age range of 1 year–46 years, 36 patients underwent surgical repair of sinus venosus ASDs and 36 patients underwent surgical repair of ostium secundum ASDs. Their data were collected and retrospectively studied from June 1, 2009, to October 1, 2016, at Ibn-Alnafees Teaching Hospital for cardiothoracic surgery in Baghdad, Iraq. We divided the patients into two groups: Group A including 36 patients who had sinus venosus ASDs and Group B including 36 patients who had ostium secundum ASDs. A comparison was made between the two groups according to preoperative variables, diagnostic modalities, intraoperative variables, and postoperative morbidity and mortality. Results: Transthoracic echocardiography (TTE) was the mainstay for the diagnosis of ASD secundum, while ASD venosus type needed more modalities for diagnosis. No operative death, postoperative bleeding, acute renal failure, SND, SVC stenosis, and PV stenosis were observed in any patient in the two groups. Residual shunt was not observed in any case in the two groups. Conclusion: TTE, transesophageal echocardiography, cardiac magnetic resonance imaging, or diagnostic catheterization may be needed for the diagnosis of sinus venosus ASD. Surgery is the mainstay of sinus venosus ASDs with low morbidity and no mortality, similar to ostium secundum ASDs.

OC65 RIGHT MINI-THORACOTOMY APPROACH FOR ATRIAL SEPTAL DEFECT CLOSURE AND PARTIAL ANOMALOUS PULMONARY VENOUS RETURN CORRECTION

Objective: Over the last 20 years, right mini-thoracotomy cardiac surgery has confirmed its feasibility and effectiveness with remarkable early and long-terms outcomes. As a consequence of the excellent results reached, interest in minimal invasive cardiac surgery is rising also in the case of grown-up congenital heart disease patients. The rational is to minimize surgical trauma, accelerate recovery, reduce the rate of wound complications and hospital-related infections, and improve cosmetic outcomes. Aim of this study is to evaluate feasibility and effectiveness of the right mini-thoracotomy approach applied to patients with diagnosis of atrial septal defect (ASD) and partial anomalous pulmonary venous return (PAPVR). Methods: Patients with ASD and PAPVR undergoing right mini-thoracotomy cardiac surgery at our Department between November 2006 and January 2018 were retrospectively reviewed. Results: During the study period 17 patients underwent ASD closure with pericardial patch and PAPVR correction with anomalous vessels addressing into left atrium through right mini-thoracotomy and retrograde arterial perfusion (Table 1). Aortic clamping was achieved through endoaortic balloon in 13 out of 17 patients (76.5%) and through trans-thoracic clamp in 4 out of 17 patients (23.5%). No cases of conversion to full sternotomy, nor intra-operative vascular complications or re-exploration for bleeding were recorded. No cases of stroke nor cases of 30-day mortality were reported. Conclusions: Right mini-thoracotomy is nowdays the standard approach for ASD closure and PAPVR correction at our Department. Results of this case series show that it is an effective and reproducible alternative to median sternotomy.

The Significance of Crochetage on the R wave of an Electrocardiogram for the Early Diagnosis of Pediatric Secundum Atrial Septal Defect.

The purpose of this study was to test the hypothesis that the incidence of crochetage on the R wave in inferior limb leads can be used for the diagnosis of pediatric secundum atrial septal defect (ASD). Two hundred fifty-six children with secundum ASD (case cohort) and 256 age- and gender-matched children without heart disease (control cohort) were included in the study. Statistical analyses were performed to test the relationship between the ASD and the crochetage on the R wave with a single lead and three leads, respectively. The impact of incomplete right bundle branch block (IRBBB) and ASD diameter (≥ 5 and < 5mm) on ASD diagnosis were also explored. Crochetage on the R wave was observed in all three inferior limb leads on 28.13% (72/256, 28 with IRBBB) of subjects with secundum ASD, while it was seen in only 2.73% (7/256, one with IRBBB) of control subjects (P < 0.001). Subgroup analysis showed that the incidence of R wave crochetage correlated with ASD size in both the single inferior limb lead (26.14%, 23/88 on ASD ≥ 5mm vs. 10.71%, 18/168 on ASD < 5mm; P = 0.001) and all three inferior limb leads (44.32%, 39/88 on ASD ≥ 5mm vs. 19.64%, 33/168 on ASD < 5mm; P < 0.001). Our findings suggest that crochetage on the R wave in inferior limb leads can serve as an independent marker for ASD diagnosis.

Intracardiac echocardiography for percutaneous patent foramen ovale and atrial septal defect occlusion.

Transesophageal echocardiography (TEE) plays aunique role in transcatheter closure of atrial septal defects (ASD) and patent foramen ovale (PFO). However, problems such as the need for general anesthesia, possible trauma from endotracheal intubation, presence of "blind spots," and occasional inadequate imaging of some cardiac structures have necessitated better imaging techniques. Our study aimed to compare the findings of TEE during the initial diagnostic examination with those from intracardiac echocardiography (ICE) acquired during the interventional procedure. Atotal of 65patients in whom TEE was used for the diagnosis of ASD or PFO were included. Of these, 40 patients (61.5%) had ASD with significant left to right shunt and 25(38.5%) patients had PFO associated with transient ischemic attack or stroke. ICE imaging was performed under local anesthesia in all patients to guide interatrial communication closure. ICE provided adequate views of the defects and surrounding structures during the various stages of device deployment. In eight patients (12.3%) an additional anatomical variation was detected. All patients had successful device implantation and were discharged 1day after the procedure. ICE is asafe and high-quality imaging technique for guiding transcatheter ASD and PFO occlusion. Additionally, ICE can both facilitate device implantation and detect cardiac abnormalities that are not identified with TEE during the initial diagnostic investigation.

Congenital and acquired Lutembacher's syndrome presenting in two adults

The coexistence of atrial septal defect (ASD) and mitral stenosis (MS) is defined as Lutembacher's syndrome (LS). LS was originally defined as the development of MS in a patient having a congenital ASD diagnosis. However, as the percutaneous interventions for MS have become widespread, the persistence of associated iatrogenic ASDs caused has given rise to a different form of the disease. LS may occur as spontaneous or iatrogenic ASD concomitant with acquired MS; this form is known as “acquired LS.” This report presents two cases illustrating congenital LS and “acquired LS.”

Long-term mortality in patients with atrial septal defect: a nationwide cohort-study.

AimsIn this nationwide cohort of atrial septal defect (ASD) patients, the largest to date, we report the longest follow-up time with and without closure in childhood and adulthood compared with a general population cohort.Methods and resultsUsing population-based registries, we included Danish individuals born before 1994 who received an ASD diagnosis between 1959 and 2013. All diagnoses were subsequently validated (n = 2277). Using the Kaplan–Meier estimates and Cox proportional hazards regression adjusted for sex, birth year, and a modified Charlson Comorbidity Index, we compared the mortality of ASD patients with that of a birth year and sex matched general population cohort. The median follow-up from ASD diagnosis was 18.1 years (range 1–53 years). Patients with ASD had a higher mortality [adjusted hazard ratio (HR): 1.7; 95% confidence interval (CI): 1.5–1.9] compared with the general population cohort. The adjusted HR 30 days after closure was 1.4 (95% CI: 1.2–1.7), and it was 2.4 (95% CI: 2.0–2.9) for patients without closure.ConclusionOverall, ASD patients had a higher long-term mortality than a general population cohort matched on birth year and gender. Our data indicate a lower relative mortality of those ASD patients undergoing closure than the ASD patients not undergoing closure.

The accuracy of ultrasound-derived three-dimensional printing models for atrial septal defect

Objective To evaluate the accuracy of three-dimensional printing atrial septal defect (ASD) models from three dimensional transesophageal echocardiography (3D-TEE) images and to lay the foundation for the application of 3D printing technology in the diagnosis and treatment of ASD. Methods Twenty patients with ASD were analyzed retrospectively.2D-TEE and 3D-TEE were performed before ASD occlusion. The 3D-TEE data were post-processed by Mimics software and the volume images of ASD in STL format were developed. Then the STL file was output and the ASD 3D models were printed. The ASD size parameters included the maximal diameter (Dmax), the minimal diameter (Dmin), circumference(C) and area(A), which were measured from 2D-TEE images, 3D-TEE images and 3D printing models, respectively. The absolute difference value of ASD size parameters between 3D printing models and 2D-TEE images, or 3D-TEE images were calculated. The sizes of occluder were recorded during the operation and the preoperative exercises on 3D printing models were performed. Results There were no significant difference in ASD size parameters among 3D printing models, 2D-TEE and 3D-TEE images(all P>0.05), the ASD size parameters were concordant well between 3D printing models and 2D-TEE or 3D-TEE images. Moreover, the absolute difference value of ASD size parameters between 3D printing models and 2D-TEE or 3D-TEE images were little. In addition, linear regression analysis revealed a significant correlation in the Dmax measured from 3D printing models and the sizes of occulder used in the operation (r=0.94, P<0.05). In the ASD 3D printing models the preoperative exercise had an impressive effect. Conclusions It is quite feasible to use 3D-TEE images as the data source of ASD 3D printing models.Ultrasound-derived ASD 3D printing models are of highly accuracy, which have the potential to provide evidence for the clinical application of 3D printing technology. Key words: Echocardiography, transesophageal; Heart septal defect, atrial; 3D printing

Status of Antenatal Detection of Congenital Heart Defects in a Northern Indian Population and Factors Influencing it

Abstract Objective To evaluate the antenatal detection rate (ADR) of congenital heart defects (CHDs) in the northern part of India and also to assess the factors affecting the same. Methods This was a prospective study performed at a tertiary referral center for pediatric cardiac care in northern India and included 200 consecutive infants (including neonates). A detailed history was obtained from the parents based on a protocol which included antenatal, birth and postnatal details including age at CHD detection. Patients with postnatal diagnosis of atrial septal defects, patent ductus arteriosus, partial anomalous pulmonary venous return, coronary and vascular ring anomalies and coarctation of aorta were excluded. Results Median age of detection of CHD was 1 mo (range = 1 d–11 mo). Fetal echocardiography (FE) was done in 23 (11.5%) patients; CHD was detected in 21 of them, giving an overall antenatal detection rate (ADR) of CHD as 10.5%. Fifteen of them were referred to a pediatric cardiologist for FE while in 8 patients, FE was performed by sonologist or fetal medicine specialist. Median gestational age at referral to a regional pediatric cardiologist for FE was 24 wk (range = 18–36 wk). Most important factor affecting the ADR was total number of ultrasounds during pregnancy, with ADR being higher in those having four or more ultrasound examinations. Conclusions Antenatal detection rate for CHD was very low. Referral for a focused fetal echocardiography during pregnancy is poor and those referred are done at very late stages of pregnancy. Measures that can help in improving the detection include following a strict protocol for cardiac screening, extending the screening to include the outflow tracts and early referral to fetal or pediatric cardiologists in case of minimal suspicion.