Modeling of multiple atrial septal defects with inferior vena cava rim deficiency: Three-dimensional visualization of the rim based on transthoracic echocardiographic imaging.

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

Human Genetics of Atrial Septal Defect.

Although atrial septal defects (ASD) can be subdivided based on their anatomical location, an essential aspect of human genetics and genetic counseling is distinguishing between isolated and familiar cases without extracardiac features and syndromic cases with the co-occurrence of extracardiac abnormalities, such as developmental delay. Isolated or familial cases tend to show genetic alterations in genes related to important cardiac transcription factors and genes encoding for sarcomeric proteins. By contrast, the spectrum of genes with genetic alterations observed in syndromic cases is diverse. Currently, it points to different pathways and gene networks relevant to the dysregulation of cardiomyogenesis and ASD pathogenesis. Therefore, this chapter reflects the current knowledge and highlights stable associations observed in human genetics studies. It gives an overview of the different types of genetic alterations in these subtypes, including common associations based on genome-wide association studies (GWAS), and it highlights the most frequently observed syndromes associated with ASD pathogenesis.

Natural History of Secundum ASD in Preterm and Term Neonates: A Comparative Study.

Atrial septal defects (ASDs) are common in neonates. Although past studies suggest ASDs  ≥ 3 mm in term neonates (TNs) are less likely to close, there is paucity of data regarding the natural history in preterm neonates (PNs), information that would inform surveillance. We sought to compare spontaneous closure rates and need for intervention for ASDs in TNs/near term (≥ 36 weeks) versus PNs (< 36 weeks). We included all TNs and PNs who underwent echocardiography at ≤ 1 month between 2010 and 2018 in our institution with an ASD ≥ 3 mm, without major congenital heart disease, and with repeat echocardiogram(s). Spontaneous resolution was defined as size diminution to < 3 mm or closure. We included 156 TNs (mean gestational age at birth 38.6 ± 1.4 weeks) and 156 PNs (29.6 ± 3.7 weeks) with a mean age at follow-up of 16 ± 19 and 15 ± 21 months, respectively (p = 0.76). Based on maximum color Doppler diameter, in TNs, ASD resolution occurred in 95% of small (3-5 mm), 87% of moderate (5.1-8 mm), and 60% of large (> 8 mm) defects; whereas, in PNs, resolution occurred in 79% of small, 76% of moderate, and 33% of large ASDs. There was a significant association between size and ASD resolution in TNs (p = 0.003), but not PNs (p = 0.17). Overall, ASD resolution rate was higher in TNs (89%) versus PNs (78%) (p = 0.009), and fewer TNs (1%) compared to PNs (7%) required ASD intervention (p = 0.02). Most ASDs identified in TNs and PNs spontaneously resolve. PNs, however, demonstrate lower ASD resolution and higher intervention rates within all size groups. These data should inform follow-up of affected neonates.

Early Post-operative ECG Changes as a Predictor of Post-pericardiotomy Syndrome Following Atrial Septal Defect Repair.

To identify risk factors associated with post-pericardiotomy syndrome (PPS) in patients undergoing surgical repair of atrial septal defects (ASD). A single-center retrospective study. Tertiary academic hospital. Included were patients of all ages who underwent surgical ASD repair, while exclusion criteria included the absence of post-operative electrocardiogram (ECG), lack of follow-up post-discharge and factors hindering ECG interpretation. Demographic and clinical data, including ECG changes indicative of pericardial inflammation, were collected. The primary outcome measure was the development of PPS, determined based on the standardized European Society of Cardiology (ESC) criteria. Among 190 patients who underwent surgical ASD repair, 154 (81%) met the inclusion criteria. Of these, 25 (16%)in total developed PPS, of which 60% were ≥ 18 years of age and 56% female. Significant associations relating both early ECG changes and pre-discharge pericardial effusion with subsequent occurrence of PPS were found in both univariate and multivariate analyses. The study establishes correlations of both early post-operative ECG changes indicative of inflammation and pre-discharge pericardial effusion with subsequent occurrence of PPS in patients undergoing surgical ASD repair. Both utilizing the standardized ESC definition of PPS and incorporating a physician-validated ECG evaluation strengthened the methodologic approach in establishing these relationships. The results also highlight the importance of considering age as a potential risk factor for PPS. Further research is needed to validate these findings and explore additional risk factors predicting early identification and management of patients at high risk for PPS following surgical ASD repairs.

Exploring Genetic Diversity of SOD2 and POU5F1 for Congenital Heart Disease in the Southwest Chinese Population.

Congenital heart disease (CHD) accounts for nearly one-third of all major congenital anomalies, with atrial septal defect (ASD) and ventricular septal defect (VSD) being the most common forms of simple CHD, which involve a large number of susceptibility genes. However, despite extensive research, the etiology of ASD and VSD remains unclear. Yunnan Province has advantages in exploring CHD pathogenesis due to its unique genetic background. Therefore, we aimed to evaluate the association between single nucleotide polymorphisms (SNPs) of genes and susceptibility to simple CHD in a specific population by means of a case-control study. A total of 337 healthy controls and 767 patients with simple CHD (501 ASD and 266 VSD) from China were recruited. Candidate SNPs were identified through whole-genome sequencing of pooled CHD patients and controls (pool-seq). Genotyping from 1,104 samples was performed, and stratified analysis was conducted to explore the association between positive SNPs and CHD subtypes. χ2 tests and logistic regression were used to analyze the relationship between each SNP and simple CHD. Of 11 SNPs identified, SOD2 rs62437333 (P = 0.005) and POU5F1 rs3130504 (P = 0.017) showed differences between the control and ASD cohorts. In the dominant inheritance model hypothesis, rs62437333 allele C carriers had increased ASD (odds ratio (OR) = 2.04, P = 0.005) and combined simple CHD risk (OR = 2.33, P = 0.012) compared to DD genotype, while rs3130504 allele C carriers had increased ASD risk (OR = 1.121, P = 0.045) compared to DD genotype.

Transcatheter versus surgical treatment for isolated superior sinus venosus atrial septal defect.

BACKGROUND: The superior sinus venosus atrial septal defect is a congenital communication between the left and right atria. Open surgical approach by patch closure has historically been the only treatment option. Recently, a transcatheter approach has been developed. This study aims to compare the efficacy and safety of surgical and transcatheter approach in treatment of sinus venosus atrial septal defect. METHODS: Between March 2010 and December 2020, 58 patients (median age: 45.4, range 14.8-73.8) underwent either surgical or transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage. RESULTS: Twenty-four patients (median age: 35.4, range 14.8-66.8) underwent surgery while 34 patients (median age: 46.8, range 15.5-73.8) had a transcatheter treatment. During the catheterization era, 41 patients was considered suitable for a transcatheter closure. In 5 patients, surgery was the patient's or referring physician's choice. In 2 cases, the procedure was unsuccessful; the remaining 34 were successfully closed (94.4% of cases). Intensive care unit stay (median of 1 day, range 0.5-4, vs. 0, range 0-2, p < 0.0001) and hospital stay (median 7 days, range 2-15 vs. 2 days, range 1-12, p < 0.0001), were significantly longer in the surgery group. Total early complication rate, consisted on procedural and in-hospital complication, were higher in the surgical group (62.5% vs. 23.5%; p = 0.005). However, complications in both groups were clinically mild. At follow-up, a small residual shunt was present in 6 patients (surgery group: 2 pts; catheterization group: 4 pts; p: NS). Imaging studies showed significant improvement of right ventricular size and unobstructed pulmonary venous return in all patients. No late complications occurred at follow-up. CONCLUSIONS: Transcatheter correction of sinus venosus atrial septal defect is effective and safe in selected patients and may be considered as a valid alternative to surgery.

A novel algorithm for classification of interatrial communications within the oval fossa in the newborn.

BACKGROUND: An interatrial communication is present in most neonates. The majority are considered the "normal" patency of the oval foramen, while a minority are abnormal atrial septal defects. Differentiation between the two with transthoracic echocardiography may be challenging, and no generally accepted method of classification is presently available. We aimed to develop and determine the reliability of a new classification of interatrial communications in newborns. METHODS AND RESULTS: An algorithm was developed based on echocardiographic criteria from 495 newborns (median age 11[8;13] days, 51.5% females). The algorithm defines three main categories: patency of the oval foramen, atrial septal defect, and no interatrial communication as well as several subtypes. We found an interatrial communication in 414 (83.6%) newborns. Of these, 386 (93.2%) were categorised as patency of the oval foramen and 28 (6.8%) as atrial septal defects.Echocardiograms from another 50 newborns (median age 11[8;13] days, 36.0% female), reviewed by eight experts in paediatric echocardiography, were used to assess the inter- and intraobserver variation of classification of interatrial communications into patency of the oval foramen and atrial septal defect, with and without the use of the algorithm. Review with the algorithm gave a substantial interobserver agreement (kappa = 0.66), and an almost perfect intraobserver agreement (kappa = 0.82). Without the use of the algorithm, the interobserver agreement between experienced paediatric cardiologists was low (kappa = 0.20). CONCLUSION: A new algorithm for echocardiographic classification of interatrial communications in newborns produced almost perfect intraobserver and substantial interobserver agreement. The algorithm may prove useful in both research and clinical practice.

Postoperative Complications, Readmissions, Lengths of Stay, and Cost Analyses of Patients Who Have Atrial Septal Defects After Total Joint Arthroplasty.

BACKGROUND: Atrial septal defects (ASDs) are a common congenital heart defect. This study aimed to determine whether patients diagnosed with ASDs undergoing total joint arthroplasty have differences in 1) medical complications, 2) readmissions, 3) lengths of stay (LOS), and 4) costs. METHODS: Using an administrative claims data set, a retrospective query from 2010 to 2020 was performed. The ASD patients were 1:5 ratio matched with controls, yielding a total of 45,695 total knee arthroplasty (TKA) (ASD = 7,635, control = 38,060) and 18,407 total hip arthroplasty (THA) (ASD = 3,084, control = 15,323) patients. Outcomes included medical complications, readmissions, LOS, and costs. Logistical regressions were used to calculate odds ratios (ORs) and P values. P values < 0.001 were significant. RESULTS: The ASD patients had higher odds of medical complications after TKA (38.8 versus 21.0%; OR 2.09; P < .001) and THA (45.2 versus 23.5%; OR 2.1; P < .001), noticeably deep vein thromboses, strokes, and other thromboembolic complications. The ASD patients were not significantly more likely to be readmitted after TKA (5.3 versus 4.7%; OR 1.13; P = .033) or THA (6.0 versus 5.7%; OR 1.05; P = .531). Patient LOS was not significantly greater in ASD patients undergoing TKA (3.2 versus 3.2 days; P = .805) but was greater after THA (5.3 versus 3.76 days; P < .001). Same-day surgery costs were not significantly increased in ASD patients after TKA ($23,892.53 versus $23,453.40; P = .066) but were after THA ($23,981.93 versus $23,579.18; P < .001). Costs within 90 days were similar between cohorts. CONCLUSION: The ASD patients have greater 90-day complications following primary total joint arthroplasty. Providers may consider preoperative cardiac clearance or adjusting anticoagulation in this population to mitigate these risks. LEVEL OF EVIDENCE: III.

Prognostic Value for Mortality of Plasma Bioactive Adrenomedullin in Patients with Pulmonary Arterial Hypertension: A Sub Analysis of the Biomarker Study in the COHARD-PH Registry.

The adrenomedullin level increases in pulmonary arterial hypertension (PAH, and correlates with a high mortality rate. Its active form, bioactive adrenomedullin (bio-ADM), has been recently developed and has significant prognostic applications in acute clinical settings. Aside from idiopathic/hereditary PAH (I/H-PAH), atrial septal defects-associated pulmonary artery hypertension (ASD-PAH) is still prevalent in developing countries and associated with increased mortality. This study aimed to investigate the mortality-wise prognostic value of the plasma bio-ADM level by comparing subjects with ASD-PAH and I/H-PAH with ASD patients without pulmonary hypertension (PH) as a control group. This was a retrospective, observational cohort study. The subjects were Indonesian adult patients who were recruited from the Congenital Heart Disease and Pulmonary Hypertension (COHARD-PH) registry and divided into three groups: (1) ASD without PH (control group), (2) ASD-PAH and (3) I/H-PAH. During right-heart catheterization at the time of diagnosis, a plasma sample was taken and assayed for bio-ADM using a chemiluminescence immunoassay. Follow-up was performed as a part of the COHARD-PH registry protocol in order to evaluate the mortality rate. Among the 120 subjects enrolled: 20 turned out to have ASD without PH, 85 had ASD-PAH and 15 had I/H-PAH. Compared to the control group (5.15 (3.0-7.95 pg/mL)) and ASD-PAH group (7.30 (4.10-13.50 pg/mL)), bio-ADM levels were significantly higher in the I/H-PAH group (median (interquartile range (IQR)): 15.50 (7.50-24.10 pg/mL)). Moreover, plasma bio-ADM levels were significantly higher in subjects who died (n = 21, 17.5%) compared to those who survived (median (IQR): 11.70 (7.20-16.40 pg/mL) vs. 6.90 (4.10-10.20 pg/mL), p = 0.031). There was a tendency toward higher bio-ADM levels in those who died among the PAH subjects, in both ASD-PAH and I/H-PAH groups. In conclusion, the plasma bio-ADM level is elevated in subjects with PAH from both ASD-PAH and I/H-PAH origins, reaching the highest levels in subjects with the I/H-PAH form. A high bio-ADM level tended to be associated with a high mortality rate in all subjects with PAH, indicating a relevant prognostic value for this biomarker. In patients with I/H-PAH, monitoring bio-ADM could represent a valid tool for predicting outcomes, allowing more appropriate therapeutical choices.

A new FDA approved stent for congenital heart disease: First-in-man experiences with G-ARMORTM.

We present the first clinical experience with a new hybrid cell structure covered stent, designed for congenital heart disease applications. It represents a significant redesign of the Cheatham Platinum (CP) Stent (Numed Inc.), maintaining the traditional benefits of the covered CP whilst significantly decreasing shortening and allowing controlled flaring at the ends through its combination of larger and standard sized cells. We first implanted the stent in 2 patients with superior sinus venosus defects with anomalous drainage of the right upper and middle lobe pulmonary veins. The first was a 40 year male and the second a 36 year old female. The third case was a 60 year old patient with near atresia of the aorta, with pre and poststenotic aortic dilation. The clinical result in all cases was excellent with no obstruction to pulmonary venous return and no visible L-R shunt on the transthoracic echo on 24 h and 2 week follow-up for the patient with sinus venosus defects and uniform complete revascularization of the aorta without any vascular complications in the patient with coarctation. These are the first uses of this stent in human subjects. The design is specifically aimed toward procedures where stent shortening is undesirable. Hence, coarctation of the aorta as well as stent implantation in preparation for percutaneous pulmonary valve placement are obvious use areas, as well as the growing body of evidence supporting percutaneous treatment of sinus venosus defects.

Iatrogenic atrial septal defect after HotBalloon ablation of atrial fibrillation.

HotBalloon-based pulmonary vein isolation (HBPVI) has yielded encouraging clinical results in the treatment of atrial fibrillation (AF). Balloon ablation requires a larger sheath, which raises the concern for a persistent iatrogenic atrial septal defect (iASD). The present study aimed to investigate the incidence, clinical features, and the predictive factors of transthoracic echocardiography (TTE)-detectable iASD after HBPVI. All patients who underwent HBPVI of AF with pre- and post-ablation TTE were retrospectively analyzed. A 17-French steerable deflectable guiding sheath was inserted into the left atrium (LA) after a transseptal puncture, and an 8-French sheath was inserted via a single transseptal hole. In a total of 190 patients, 98 (52%) paroxysmal AF (PAF) and 92 (48%) non-PAF, the iASD was detected in 18 (9.4%) with a mean follow-up period of 12.7 ± 2.5 months after HBPVI. All patients had no clinical symptoms related to iASD. No embolic or heart failure events occurred. In the multivariate analysis, LA volume index and LA procedure time were identified as significant independent predictors of iASD. After HBPVI, TTE-detectable iASD was found in 9.4% of study patients. Larger LA size and longer LA procedure time were predictive factors for the persistence of iASD. All patients with iASD had no clinical symptoms 12 months after HBPVI; however, long-term follow-up may be necessary.

Midazolam for conscious sedation in transcatheter device closure of atrial septal defects guided solely by transthoracic echocardiography.

OBJECTIVES: To investigate the safety and feasibility of midazolam for conscious sedation in transcatheter device closure of atrial septal defects guided solely by transthoracic echocardiography. METHODS: A retrospective analysis was performed on 55 patients who underwent transcatheter device closure of atrial septal defects from October, 2019 to May, 2020. All patients received intravenous midazolam and local anesthesia with lidocaine to maintain sedation. A group of previous patients with unpublished data who underwent the same procedure with general anesthesia was set as the control group. The relevant clinical parameters, the Ramsay sedation scores, the numerical rating scale, and the post-operative satisfaction questionnaire were recorded and analyzed. RESULTS: In the midazolam group, the success rate of atrial septal defect closure was 98.2%. Hemodynamic stability was observed during the procedure. None of the patients needed additional endotracheal intubation for general anesthesia. Compared with the control group, the midazolam group had no statistically significant differences in the Ramsay sedation score and numerical rating scale scores. Patients in the midazolam group experienced more post-operative satisfaction than those in the control group. CONCLUSIONS: Conscious sedation using midazolam is a safe and effective anesthetic technique for transcatheter device closure of atrial septal defects guided solely by transthoracic echocardiography.

Identification and functional study of GATA4 gene regulatory variants in atrial septal defects.

BACKGROUND: Congenital heart disease (CHD) is the leading cause of mortality from birth defects. In adult CHD patients with successful surgical repair, cardiac complications including heart failure develop at late stage, likely due to genetic causes. To date, many mutations in cardiac developmental genes have been associated with CHD. Recently, regulatory variants in genes have been linked to many human diseases. Although mutations and splicing variants in GATA4 gene have been reported in CHD patients, few regulatory variants of GATA4 gene are identified in CHD patients. METHODS: GATA4 gene regulatory region was investigated in the patients with atrial septal defects (ASD) (n = 332) and ethnic-matched controls (n = 336). RESULTS: Five heterozygous regulatory variants including four SNPs [g.31360 T>C (rs372004083), g.31436G>A, g.31437C>A (rs769262495), g.31487C>G (rs1053351749) and g.31856C>T (rs1385460518)] were only identified in ASD patients. Functional analysis indicated that the regulatory variants significantly affected the transcriptional activity of GATA4 gene promoter. Furthermore, two of the five regulatory variants have evidently effected on transcription factor binding sites. CONCLUSIONS: Our data suggested that GATA4 gene regulatory variants may confer ASD susceptibility by decreasing GATA4 levels.

Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Secundum atrial septal defect (ASD) is the most common adult congenital heart defect and can present with wide variation in clinical findings. With the intention of preventing morbidity and mortality associated with late presentation of ASD, consensus guidelines have recommended surgical or percutaneous ASD closure in adults with right heart enlargement, with or without symptoms. The aim of the present analysis was to determine if the protective effect of secundum ASD closure in adults could be qualified by pooling data from published studies. A systematic review and meta-analysis were performed by using EMBASE, MEDLINE (through PubMed), and the Cochrane Library databases to assess the effect of secundum ASD percutaneous or surgical closure in unoperated adults ≥18 years of age. Data were pooled across studies with the DerSimonian-Laird random-effects model or a Bayesian meta-analysis model. Between-study heterogeneity was assessed with Cochran's Q test. Bias assessment was performed with the Newcastle-Ottawa Scale and the Cochrane Risk of Bias Tool, and statistical risk of bias was assessed with Begg and Mazumdar's test and Egger's test. A total of 11 nonrandomized studies met the inclusion criteria, contributing 603 patients. Pooled analysis showed a protective effect of ASD closure on New York Heart Association functional class and on right ventricular systolic pressure, volumes, and dimensions. Two additional studies comprising 652 patients were reviewed separately for mortality outcome and primary outcome of interest because they did not meet the inclusion criteria. Those studies showed that ASD closure was associated with a weak protective effect on adjusted mortality rate but no significant impact on atrial arrhythmias in patients >50 years of age. Across all studies, there was significant heterogeneity between studies for nearly all clinical outcomes. The overall body of evidence was limited to observational cohort studies, the limitations of which make for low-strength evidence. Even within the parameters of the included studies, quality of evidence was further diminished by the lack of well-defined clinical outcomes. In conclusion, pooled data analysis on the impact of secundum ASD closure in adults was notably limited because of the lack of randomized controlled trials in patients with only secundum ASD. The few cohort studies in this population demonstrated improvement in functional status and right ventricular size and function as shown by echocardiogram. However, our findings suggest that at the time of this publication, insufficient data are available to determine the impact of ASD repair on mortality rate in adults.

Transcatheter closure of sinus venosus atrial septal defect with anomalous pulmonary venous drainage: Innovative technique with long-term follow-up.

INTRODUCTION: Surgical closure of sinus venosus atrial septal defect (SVASD) is the standard management. A safe and effective transcatheter approach will be an attractive option. OBJECTIVES: To assess the feasibility and long-term safety of transcatheter closure of SVASD with anomalous pulmonary venous drainage. PATIENTS AND METHODS: From July 2011 to October 2013, four patients with large SVASD and anomalous right upper pulmonary venous (RUPV) drainage underwent transcatheter closure of their defects at Ibn-Albitar Center for Cardiac Surgery, Baghdad, Iraq. Two patients with superior vena cava (SVC)-type SVASD underwent closure using covered Cheatham-Platinum (CP) stents with no need for septal occluder. The other two patients had large RA-type SVASD who underwent closure using covered CP stents only in one patient and stents and device in the other one. An angiogram in the RUPV during balloon inflation in the SVC was done to ensure that the RUPV drains back to the left atrium. The covered CP stent was mounted and hand crimped onto Z-Med™ or BIB-balloon catheters and deployed in the desired location under transesophageal echocardiography guidance. RESULTS: The two patients with SVC-type SVASD underwent successful closure using two overlapping covered CP stents implanted in the SVC, thus creating total septation between the SVC and the RUPV. The RA-type SVASD patients underwent closure using two overlapping covered CP stents. One with mild to moderate residual shunt that completely disappeared at 12 months follow-up after implantation of a second 45 mm CP stent. A significant residual shunt in the second patient was closed successfully using a PFO device. CONCLUSION: Transcatheter closure of SVASD through SVC stent insertion with or without subsequent device implantation is feasible and effective.

The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry: a descriptive study from single-center hospital registry of adult congenital heart disease and pulmonary hypertension in Indonesia.

BACKGROUNDS: The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry is the first registry for congenital heart disease (CHD) and CHD-related pulmonary hypertension (PH) in adults in Indonesia. The study aims to describe the demographics, clinical presentation, and hemodynamics data of adult CHD and CHD-related PH in Indonesia. METHODS: The COHARD-PH registry is a hospital-based, single-center, and prospective registry which includes adult patients with CHD and CHD-related PH. The patients were enrolled consecutively. For this study, we evaluated the registry patients from July 2012 until July 2019. The enrolled patients underwent clinical examination, electrocardiography, chest x-ray, 6-min walking test, laboratory measurement, and transthoracic and transesophageal echocardiography. Right heart catheterization was performed to measure hemodynamics and confirm the diagnosis of pulmonary artery hypertension (PAH). RESULTS: We registered 1012 patients during the study. The majority were young, adult females. The majority of CHD was secundum ASD (73.4%). The main symptom was dyspnea on effort. The majority of patients (77.1%) had already developed signs of PH assessed by echocardiography. The Eisenmenger syndrome was encountered in 18.7% of the patients. Based on the right heart catheterization, 66.9% of patients had developed PAH. Patients with PAH were significantly older, had lower peripheral oxygen saturation, had lower 6-min walking distance, and higher NTproBNP. The NTproBNP level independently predicted the development of PAH among CHD. CONCLUSIONS: The COHARD-PH registry is the first Indonesian adult-CHD and CHD-related PH registry. The demographics, clinical presentation, and hemodynamics dataof this registry reflect the situation in developing countries which needs to be compared with similar registries from developed countries.

Tricuspid Regurgitation in Ostium Secundum Atrial Septal Defects: Repair or Not?

BACKGROUND: Longstanding ostium secundum atrial septal defects lead to functional tricuspid regurgitation. Significant functional tricuspid regurgitation associated with left heart valve disease is addressed at the time of primary left heart valve surgery. In contrast, there is no global recommendation for tricuspid regurgitation associated with atrial septal defects. This study assesses changes in tricuspid regurgitation after isolated atrial septal defect closure. METHODS: Retrospectively, records were examined of 100 patients who underwent isolated ostium secundum atrial septal defect closure without tricuspid valve repair. Echocardiograms were done preoperatively and 3 days, 3 months, and 1 year after surgery. Data on tricuspid regurgitation status, right ventricle dimensions, and pulmonary artery hypertension status were collected and analyzed. RESULTS: After surgical closure, echocardiography showed a regression of tricuspid regurgitation to mild or less in 76% of patients at 3 days, 89% at 3 months, and 93% at 1 year. Severe pulmonary artery hypertension (32% patients preoperatively) showed statistically significant regression: 14% at 3 days, 10% at 3 months, and 2% at 1 year. Preoperatively, the mean right ventricular internal diameter was 37.9 mm, which decreased to 34 ± 5.5 mm (mean ± standard deviation) at 3 days, 32.3 ± 5.3 mm at 3 months, and 31.3 ± 5.4 mm at 1 year. It was also noted that regression favored patients who were <25 years old. CONCLUSION: Tricuspid valve repair may not be required in patients with ostium secundum atrial septal defect with functional tricuspid regurgitation.

Maternal risk factors for congenital heart defects in infants with Down syndrome from Western Mexico.

Atrioventricular septal defects (AVSDs) have been identified as intriguingly infrequent among Hispanics with Down syndrome (DS) born in the United States. The aim of this study was to evaluate the effect of possible maternal risk factors in the presence of congenital heart defects (CHDs) in Mexican infants with DS. A total of 231 live birth infants born with DS during 2009-2018 at the "Dr. Juan I. Menchaca" Civil Hospital of Guadalajara (Guadalajara, Mexico) were ascertained in a case-control study. Patients with DS with any major CHD were included as cases and those without major CHD as controls. Potential risk factors were analyzed using logistic regression. Of eligible infants with DS, 100 (43.3%) had ≥1 major CHDs (cases) and were compared with a control group of 131 infants (56.7%) with DS without CHDs. Prevalent CHDs were ostium secundum atrial septal defects (ASDs) (46.9%), ventricular septal defects (27.3%), and AVSDs (14%). Lack of folic acid supplementation before pregnancy had a significant risk for CHDs in infants with DS (adjusted odds ratio [aORs] = 2.9 (95% confidence interval [95% CI]: 1.0-8.6) and in the analysis by subtype of CHDs, also, for the occurrence of ASDs (aOR = 11.5, 95% CI: 1.4-94.4). Almost half of the infants with DS in our sample had CHDs, being ASD the commonest subtype and AVSD the rarest. Our ethnic background alone or in concomitance with observed nutritional disadvantages seems to contribute differences in CHD subtype rates in our DS patients.

Biventricular contractility during exercise in adults with small, unrepaired atrial septal defects.

BACKGROUND: Adult patients with a small, unrepaired atrial septal defect (ASD) have higher mortality and increased risk of several comorbidities compared to general population. Further, reduced submaximal and peak exercise capacity was recently demonstrated in these patients. The mechanisms behind these findings remain unexplained and, therefore, biventricular contractility during exercise was assessed by evaluating the force-frequency relationship in the same group of patients. METHODS: Adults patients with a small, unrepaired ASD and healthy age- and gender-matched controls were examined using echocardiography during supine bicycle exercise. Continuous tissue velocity Doppler was used to evaluate isovolumetric acceleration (IVA) and systolic velocities during an incremental workload protocol. All data were analyzed post hoc in a blinded fashion. RESULTS: We included 30 patients previously diagnosed with a small, unrepaired ASD (mean age 35 years, 63% female) and 25 controls (mean age 34 years, 64% female). Patients had similar values of IVA and systolic velocities at rest when compared with the healthy controls. Further, no differences in IVA was found at peak heart rate for neither the left ventricle (90 ± 39 vs 129 ± 68 cm/s2 , P = 0.1547) nor the right ventricle (128 ± 56 vs 154 ± 56 cm/s2 , 0.5691). There were no correlations between peak velocities and the lower exercise capacity previously reported in these patients. CONCLUSION: Adult patients with a small, unrepaired ASD have normal biventricular contractility at rest and during exercise when compared with healthy peers. Consequently, the pathophysiological mechanisms behind the impaired exercise capacity previously demonstrated in these patients remains unknown and will be a target for future work.

Use of Pulmonary Inhalants Remains Remarkably High After Atrial Septal Defect Closure.

BACKGROUND: Post-repair atrial septal defects (ASD) patients are frequently discharged from follow-up, but the extent of pulmonary symptoms long-term post-repair is unknown. Methods and Results: The national CONgenital CORvitia registry was linked to the national Drug Registry to investigate all ambulatory-dispensed pulmonary inhalants for 2006-2014. ASD patients were compared with age- and sex-matched referents from the general population. A total of 1,959 adult patients (age 42±17 years; 66% female; 1,223 [62%] repaired) were included. Compared with the referents, ASD patients had more inhalant use, even at long-term post-repair follow-up (OR=1.81 [95% CI 1.62-2.03]; P<0.001). CONCLUSIONS: ASD patients had 2-fold higher inhalant use compared with referents even at long-term post-repair follow-up, suggesting persistent pulmonary functional impairment.