Search

Lin, Tian, Huaping Jia, Yunming Li, Yongxing Xu, Bei Zhao, Dong Zheng, Hongfeng Yan, Meihui Zhao, Yanlei Li, Liping Xia, Fengxia Zhou, Cuiping Liu, Ke Ma, Ma Mi, and Jianwen Gu. Epidemiological survey of congenital heart disease among children aged from 2 to 18 in Suo County, Nagqu, Tibet. High Alt Med Biol . 00:000-000, 2024. Background: Studies have reported the prevalence of congenital heart disease (CHD) in parts of Tibet, but relative epidemiological surveys are rare. We aimed to explore the prevalence of CHD in children and its relationship with family history in Suo County, Nagqu, Tibet, an altitude of 3,980 meters. Methods: We recruited 4,002 children aged 2-18 years. Subjects underwent a family history investigation, cardiac auscultation, and clinical manifestation examination and then received echocardiographic screening. Results: The prevalence of CHD among children in Suo County was 0.97% (39 cases), much higher than the prevalence at sea level. The most common subtype was atrial septal defect, accounting for 53.9% of CHD, followed by patent ductus arteriosus (33.3%) and ventricular septal defect (12.8%). We also found that children whose mothers had previously borne children with CHD had a higher risk of CHD than those without ( p = 0.002); other factors related to CHD during pregnancy, such as smoking, drinking, drug use, and viral infection, showed no statistical differences between children with and without CHD. Conclusions: The prevalence of CHD in children in Suo County is much higher than at low altitude, consisting mostly of simple forms with left-to-right shunt, with rare complex CHD. These results support implementing diagnostic and treatment plans to prevent CHD in Suo County.

Objectives: Paradoxical embolism from right-to-left shunt through atrial septal defect (ASD) and patent foramen ovale (PFO) is a well-accepted cause of "cryptogenic" strokes (CS). To better understand the pathogenic role of ASD, we compared ASD patients with CS having a high and low likelihood of being PFO-related., Methods: In the Acute Stroke Registry and Analysis of Lausanne, we calculated prevalence of PFO and ASD in CS patients undergoing echocardiography, and calculated odds ratios (OR) when compared to non-CS. Using the Risk of Paradoxical Embolism (RoPE) score, we divided CS PFO patients in high (HL-PFO, RoPE 8-10) and low-likelihood (LL-PFO, RoPE 0-4) PFO-related stroke. We then performed univariate comparison of epidemiological, clinical and radiological variables of ASD patients with both PFO groups., Results: Among all CS, prevalence of ASD and PFO were 1.3% and 36.8% respectively. When compared to non-CS, ASD and PFO were associated with CS (OR of 5.2, CI= 1.6-16.6, and 2.8, CI= 2.1-3.8). Compared with HL-PFO, ASD patients were older, more often female, had more cardiovascular risk factors and silent strokes. Compared with LL-PFO, ASD patients were younger, more often female, and had less risk factors. No differences were found for clinical and radiological characteristics and clinical outcome., Conclusion: ASD is a rare stroke risk factor for CS. Since characteristics of such patients lie in-between high and low-likelihood paradoxical PFO-strokes, a thorough work-up for other stroke mechanisms is warranted. Individual evaluation of the likelihood of the ASD being causative for stroke may be preferable over routine ASD closure., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Michel Patrik reports financial support was provided by Swiss Heart Foundation., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Patent foramen ovale (PFO) and atrial septal defects (ASD) have been described in up to 30 % of subjects in autopsy series but contemporary data are scarce. It is important to confirm the prevalence of ASD/PFO in the general population given the potential associated stroke risk and the increasing availability of intervention via PFO closure., Methods: A state-wide prospective out-of-hospital cardiac arrest registry (OHCA) identified all patients aged 1 to 50 years who experienced OHCA in Victoria, Australia from April 2019 to April 2022 and subsequently underwent autopsy with a cardiac cause of death identified. Autopsy was performed including visual description of any ASD and identification of probe patency of foramen ovale., Results: A total of 517 patients underwent autopsy in the setting of sudden cardiac death; 36 patients (6.9 %) had a probe-patent foramen ovale, 2 patients (0.4 %) had secundum ASD, and 2 patients (0.4 %) had both a PFO and ASD (1 of whom had undergone percutaneous repair of both lesions). Twelve patients (2.3 %) had a prior history of cerebrovascular accident either recorded on medical history or detected on neuropathological examination; however none of these patients had a PFO or ASD., Conclusions: The combined rate of PFO and ASD in a cohort of 517 patients undergoing autopsy was 7.9 %. None of these patients had experienced a cerebrovascular accident. This rate of PFOs appears lower than earlier reports and raises the possibility that the relative risk of an associated stroke could be higher than previously estimated., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: The left atrial septal pouch (LASP) is a small anatomical septal recess in the heart that has been linked with cardioembolic events. A systematic appraisal of the existing literature is necessary to establish a better understanding of the risk as studies continue to indicate a correlation between LASPs and cryptogenic strokes., Objectives: To determine the level of association between the presence of LASP and the risk of developing cryptogenic stroke., Methods: We searched PubMed, EMBASE and Scopus for studies comparing the prevalence of LASP in patients with cryptogenic stroke against non-cryptogenic stroke control groups from inception till December, 2023. The Newcastle Ottawa scale was used for quality assessment and Comprehensive Meta-Analysis Version 3.3 was used for data analysis with odds ratio (OR) as the effect measure., Results: Our review included a total of 10 retrospective, observational studies published between 2010 to 2022. A total of 683 cases of cryptogenic strokes were identified, out of which 33.1 % (n = 271) were associated with a LASP. Among the non-cryptogenic stroke controls (n = 2641), LASP was present in 20.6 % cases (n = 476). The aggregate OR for cryptogenic stroke was 1.618 times greater than non-cryptogenic stroke (p < 0.001) among LASP cases, CONCLUSION: The presence of a septal pouch in the left atrium is significantly linked to a higher risk of developing cryptogenic strokes. As a potential site of thrombus formation and subsequent dislodgement, further large-scale studies are necessary to establish the guidelines for management and prophylaxis to prevent embolic events., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Inc.)

Atrial septal defect secundum is a common type of congenital heart defect and even more common among children born premature. The aim of this study was to assess premature birth as a potential associated risk factors for cardiac morbidity in children with isolated ASD II. In this retrospective national registry-based case-control study all children born in Sweden between 2010 and 2015 with an isolated ASD II diagnosis were included. Association between premature birth and cardiac morbidity in children with isolated ASD II was assessed by different outcomes-models using conditional logistic regression and adjustments were made for confounding factors. Overall, 11% of children with an isolated ASD II received treatment for heart failure. Down syndrome was the only independent risk factors for associated with cardiac morbidity in children with ASD II (OR = 2.25 (95%CI 1.25-4.07). Preterm birth in children was not associated with an increased risk of ASD II cardiac morbidity., (© 2024. The Author(s).)

Background: Cryptogenic stroke, whose underlying pathology is unknown, accounts for 30-40% of all ischemic strokes. Studies have mentioned the association between atrial septal abnormalities and cryptogenic stroke, but there are still disparities in the results among different studies., Objective: We aimed to clarify the prevalence of atrial septal abnormalities in patients with cryptogenic stroke., Methods: We conducted a cross-sectional study on 91 patients with cryptogenic stroke/transient ischemic attack from March 2021 to March 2022. We evaluated the demographic data of the patients and also the existence of neurologic attacks. Furthermore, echocardiography was performed to determine the type of atrial septal abnormality., Results: Out of 91 patients with cryptogenic stroke/transient ischemic attack, 16 patients (17.5%) had patent foramen ovale, 1 man (1.1%) had atrial septal aneurysm, and 1 woman (1.1%) had an atrial septal defect. Patients with patent foramen ovale were significantly younger than those without. The size of patent foramen ovale in patients with cryptogenic stroke was larger than those with transient ischemic attack, but this difference was not significant. Also, the size of the patent foramen ovale (length and width) was not significantly related to any of the demographic variables (p-value = 0.544, 0.604)., Conclusion: Based on our results, the prevalence of atrial septal abnormalities was relatively high. Considering these issues and the importance of preventing neurological accidents in patients, especially young people, it is recommended to always consider atrial septal disorders and, if diagnosed, to carry out the necessary treatment in this field., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Background . It has been unclear whether simple atrial septal defect (ASD) is an independent risk factor for infective endocarditis (IE). This study aimed to untangle the risk of endocarditis in a large nationwide cohort. Methods . We acquired data from the Finnish hospital discharge register on all individuals with ASD diagnosis from 1969 to 2019. Patients with complex congenital cardiac abnormalities were ruled out. Five individualized controls from the general population were matched to the ASD patient's birth year, sex, and residence at the index date. All the patients with ICD-8, -9, or -10 diagnosis codes for IE were gathered from the hospital discharge registry. Results . Altogether, 8322 patients with ASD and 39,237 individualized controls were enrolled in the study. Median follow-up was 21.6 years (IQR 11.8-36.9) from the first hospital contact. In total, 24 (16 male) cases of infective endocarditis among ASD patients and 10 (8 male) cases among controls were diagnosed during the follow-up. The incidence of endocarditis was 0.11 per 1000 person-years in the patients with ASD and 0.011 per 1000 person-years in the controls. The adjusted risk ratio for endocarditis was 13.51 (95% CI: 6.20-29.46) in patients with ASD compared to the control cohort. Patients with ASD and endocarditis had higher long-term mortality than individualized control patients (MRR 2.25, 95% CI: 1.23-4.11). Conclusions . The incidence of IE in patients with ASD was higher than in the general population. Mortality associated with IE was higher in patients with ASD compared to controls.

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

Background: This study examined risk for developmental disabilities in preschool-aged children with a congenital heart defect (CHD) at the population level., Methods: Statewide birth, birth defects, and preschool developmental disability records were integrated. The final sample included 1,966,585 children (51.0% male). Children were grouped by type(s) of CHD: critical CHD, noncritical CHD, atrial septal defect, or no major birth defects (groups were mutually exclusive)., Results: Children with a CHD (any type) were at increased risk for developmental disability (any type) (RR 2.08, 95% CI 2.03-2.14, P < .001). Children in the critical CHD, noncritical CHD, and atrial septal defect groups were at increased risk for developmental delay, intellectual disability, language impairment, other health impairment, and any disability. Children in the atrial septal defect group were at increased risk for autism spectrum disorder and speech impairment. For all CHD groups, risk was greatest for other health impairment and intellectual disability., Conclusions: Increased risk for developmental disabilities was identified for children with less severe CHDs as well as for children with more severe (critical) CHDs. All children with CHDs should be closely monitored so that appropriate interventions can be initiated as early as possible to maximize learning outcomes., (© 2023 The Authors. Birth Defects Research published by Wiley Periodicals LLC.)

Patients who underwent transcatheter edge-to-edge repair (TEER) or transcatheter mitral valve replacement (TMVR) have a transeptal access created by an iatrogenic atrial septal defect (ASD) which leads to significant complications requiring closure. Given limited data, we used the National Inpatient Sample between 2015 and 2020 to evaluate the clinical outcomes of percutaneous closure of ASD (PC-ASD) in TEER/TMVR hospitalizations. A total of 44,065 eligible weighted hospitalizations with either TEER (n = 39,625, 89.9%) or TMVR (n = 4,440, 10.1%) with a higher rate of PC-ASD in the TMVR group (10.7% vs 2.0%, p <0.01). The TEER with PC-ASD group were more likely to experience acute heart failure and right ventricular failure and had longer hospital stays but there was no difference in in-hospital mortality compared with the no PC-ASD group. In the TMVR group, there was no difference in the odds of acute heart failure, right ventricular failure, cardiogenic shock, or acute hypoxic respiratory failure, but the odds of mechanical circulatory support, in-hospital mortality, and length of stay were significantly higher in patients with PC-ASD in the TMVR group. In conclusion, rates of percutaneous closure of ASD after TEER were lower than after TMVR and associated with worse in-hospital mortality in TMVR but not in TEER. Further prospective clinical trials are needed to identify patients who would benefit from the closure of iatrogenic ASD., Competing Interests: Declaration of Competing Interest The authors have no competing interests to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Congenital heart defects (CHDs) are shown to have an association with maternal diabetes mellitus. The Bahraini population has a high prevalence of diabetes 16.3% thus putting it at increased risk of developing CHDs in infants of diabetic mothers (IDMs)., Objective: Describing the prevalence of CHDs in IDM in the Kingdom of Bahrain., Design: A retrospective clinical study., Setting: Bahrain Defense Force Hospital, Kingdom of Bahrain., Methods: The study took place from January 1998 to January 2020. A history was recorded for all patients who were referred to the only tertiary cardiac center in Bahrain for echocardiography. Data was recorded on an Excel Sheet for analysis. A cardiac anatomy survey was conducted by an experienced pediatric cardiologist for each patient and the defects were categorized into acyanotic and cyanotic lesions., Results: Five thousand five hundred sixty-nine patients were referred for cardiac echocardiography. Three thousand two hundred fifty-six patients were diagnosed with CHDs, 2,987 were non-IDM whereas 269 were IDM. Patients diagnosed with non-structural defects were excluded. Atrial septal defect (ASD) was identified in 744 patients and was more likely to occur in non-IDM (p-value = .005). Hypertrophic obstructive cardiomyopathy (HOCM) was identified in 35 patients and was more likely to occur in IDM (p-value < .001). Transposition of the great arteries (TGAs) was identified in 80 patients and was more likely to occur in IDM (p-value .002). Double inlet left ventricle (DILV), Hypoplastic Left Heart Syndrome (HPLHS), and Other Uni-Ventricular Hearts were all more likely to occur in IDM with p-values < .05., Conclusion: This study showed significant association between fetal exposure to diabetes and the development of ASD, HOCM, TGA, DILV, HPLHS, and Other Uni-Ventricular Hearts., (© 2023 Wiley Periodicals LLC.)

Background: This study aimed to collect and analyze the literature data regarding Chiari network (CN) and other right atrium (RA) remnants comprising the Eustachian and Thebesian valves (EV, ThV) as a potential entrapment site during different percutaneous cardiac procedures (PCP)., Methods and Results: A systematic search was conducted using Pubmed and Embase databases following the PRISMA guidelines to obtain available data concerning PCP associated with entrapment of inserted materials within CN-EV-ThV. The final analysis included 41 patients who underwent PCP with reported material entrapment within these RA remnants. The PCP was atrial septal defect (ASD)/patent foramen ovale (PFO) closure, catheter ablation, and pacemaker/defibrillator implantation in 44%, 22%, and 17% of patients, respectively. The entrapped materials were ASD/PFO devices, multipolar electrophysiology catheters, passive-fixation pacing leads, and J-guidewires in about 30%, 20%, 15%, and 10% of patients, respectively. Intraprocedural transthoracic, transoesophageal and intracardiac echocardiography showed sensitivity to reveal these structures of 20%, ∼95%, and 100%, respectively. A percutaneous approach successfully managed 70% of patients, while cardiovascular surgery was required in 20% and three patients died (7.3%)., Conclusions: CN and other RA remnants may cause entrapment of various devices or catheters during PCP requiring right heart access. The percutaneous approach, guided by intraprocedural imaging, appears safe and effective in managing most patients. Prevention includes recognizing these anatomical structures at baseline cardiac imaging and intraprocedural precautions. Further studies are needed to analyze the actual incidence of this condition, its clinical impact and appropriate management., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Objective: To determine the frequency of subtypes of Down syndrome by karyotyping, and to establish the frequency of congenital cardiac defects in this population., Methods: The cross-sectional study was conducted at the Department of Genetics, Children Hospital, Lahore, Pakistan, from June 2016 to June 2017, and comprised of Down Syndrome patients aged <15 years. They were subjected to karyotypic analysis for determining the subtype of the syndrome, and echocardiography of all cases was done for the assessment of congenital cardiac defects. The two findings was subsequently used to establish a relation between the subtypes and congenital cardiac defects. Data collected, entered and analyzed by the SPSS version 20.0., Results: Among the 160 cases, trisomy 21 was found in 154(96.2%), translocation 5(3.1%) and mosaicism 1(0.6%). Overall, 63(39.4%) children had cardiac defects. Among such patients, patent ductus arteriosus was most common 25(39.7%), followed by ventricular septal defects24(38.1%), atrial septal defects16(25.4%), complete atrioventricular septal defects 8(12.7%), and Tetralogy of Fallot3(4.8%), while 6(9.5%) children had other defects. Atrial septal defects was the most common double defect 9(56.2%) and had the highest coexistence with patent ductus arteriosus in Down syndrome cases with congenital cardiac defects., Conclusions: In Trisomy 21, the most common cardiac defect was patent ductus arteriosus, followed by ventricular septal defects in isolated defects, whereas in mixed defects, atrial septal defects and patent ductus arteriosus were the highest.

Introduction: Atrial septal defect (ASD) is a common congenital heart disease in children that uncommonly presents with pulmonary hypertension. Much is not known about the exact predictor of PAH in children with ASD., Objectives: This study aimed to determine the predictors of pulmonary hypertension in children with ASD., Patients and Methods: This was a descriptive analysis of children with ASD carried out in three different institutions over a five-year period. Data entry and analysis were done using IBM Statistical Package for Social Sciences (SPSS) statistical software, version 25., Results: The majority of the participants, 52.2%, had pulmonary hypertension and 62.5% of them occurred as mild pulmonary hypertension. There was a very weak positive correlation between pulmonary hypertension and the size of atrial septal defect, increases in size of atrial septal defect correlate with increases in pulmonary hypertension and this was found not to be statistically significant (n = 67, r = 0.193, p = 0.118). There was a positive correlation between the size of atrial septal defect and the age of participants in months, increases in age correlate with increases in size of atrial septal defect and this was found to be statistically significant (n = 67, r = 0.357, p = 0.003).The highest proportion of respondents who had pulmonary hypertension, 64.7%, was seen among children less than 1 year old while the least proportion, 27.3%, was within 1-5 years, and the difference in proportions was found to be statistically significant (χ 2  = 8.187, p = 0.017)., Conclusion: Pulmonary hypertension in children with ASD occur usually in the mild form. Age is the only strong predictor of PAH in children with isolated ASD.

Background: Patients with interatrial shunts (patient foramen ovale/atrial septal defect) are potentially at increased risk for paradoxical air embolism following computed tomography (CT) scans with intravenous (IV) contrast media injection. IV in-line filters aim to prevent such embolisms but are not compatible with power injection required for diagnostic CT., Purpose: The purpose of this study was to determine whether the incidence of paradoxical embolism to the heart and brain in patients with an interatrial shunt is higher compared with controls within 48 hours following injection of IV contrast media without IV in-line filter., Methods: This is a retrospective cohort study conducted at a large tertiary academic center, which included a total of 2929 consecutive patients who underwent 8983 CT scans with IV contrast media injection between July 1, 2000 and April 30, 2018. Diagnosis of an interatrial shunt was confirmed by transthoracic or transesophageal echocardiography. Incidence and risk of cardiac embolic events (new troponin elevation, >0.1 ng/mL) and neurological embolic events (new diagnosis of stroke/transient ischemic attacks) were evaluated., Results: Among the 2929 patients analyzed (mean±SD age, 61±14 y), 475/2929 (16.2%) patients had an interatrial shunt. After applying the exclusion criteria, new elevated troponin was found in 8/329 (2.4%; 95% confidence interval [CI]: 1.1-4.7) patients with an interatrial shunt compared with 25/1687 (1.5%; 95% CI: 0.9-2.2) patients without an interatrial shunt. New diagnosis of stroke occurred in 2/169 (1%; 95% CI: 0.3-4.2) of patients with an interatrial shunt compared with 7/870 (0.8%; 95% CI: 0.4-1.7) without interatrial shunt., Conclusion: Among patients with echocardiographic evidence of an interatrial shunt, IV CT contrast administration without an in-line filter does not increase the incidence of cardiac or neurological events., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background Device closure of atrial septal defect (ASD) has emerged as a treatment modality for the past 3 decades and has changed the natural history of ASD compared to that of surgical closure. Early intervention in ASD retards the geometrical and electrical remodelling of the atrium that contributes to the development of atrial tachyarrhythmias. We studied the incidence of atrial arrhythmias in patients undergoing surgical and device closure of ASD. Methods We did this retrospective observational study at a tertiary referral centre, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala. Patients of all age groups undergoing surgical and device closure of ASD between 1 January 2003 and 31 December 2008 were included to compare the incidence and nature of atrial arrhythmias and also analyse the difference in new-onset atrial arrhythmias between the two arms. Results Of 277 patients, 144 with surgical closure and 133 with device closure were followed up for 10-15 years. A larger number of men underwent surgical closure (41.7%) compared to device closure (25.6%). The mean (SD) follow-up was 12.6 (3.7) years in the surgical closure group and 10.9 (2.6) years in the device closure arm. There were a larger number of patients with atrial tachyarrhythmias in the surgical closure group (6.3%) compared to the device closure group (0.8%) at baseline (p=0.02). A significantly larger number of patients had atrial fibrillation in the surgical closure group (5.6%) compared to the device closure group (0.7%) at baseline (p=0.003). Pulmonary hypertension at baseline was present in 38.9% of patients in the surgical closure group and in 23.3% of patients in the device closure group (p=0.006). New-onset atrial arrhythmias occurred in patients ≥30 years of age (p=0.006) and exclusively in patients with pulmonary hypertension in the surgical group (3.7%) and in the device closure group (6.6%). This was statistically significant in the device closure group (p=0.05) but not in the surgical closure group (p=0.13). The incidence of new-onset arrhythmias was not statistically significant in both groups. Conclusions Atrial arrhythmias were significantly more common in patients who underwent surgical or device closure at ≥ 30 years of age and in patients with pulmonary hypertension. There was no difference in new-onset atrial arrhythmias between the surgical and device closure groups. Our study results suggest that surgical or device closure before 30 years of age and before the development of atrial arrhythmias may be beneficial with respect to the development of atrial arrhythmias.

Background: Congenital heart diseases (CHD) are the most important congenital anomaly. The burden and types are important for health planning and preparation for surgery. This study was conducted to determine the prevalence, anatomic types and defect sizes of the CHDs seen in a tertiary hospital., Methods: A retrospective review of echocardiographic records of children seen in a tertiary centre over a twelve-year period was conducted. Using information from the echocardiograms, the anatomic types and defect sizes of the CHDs were determined using known classifications. Statistical analysis was done using IBM-SPSS version 21., Results: The prevalence of CHD was 10.3/1000 children with a M:F ratio of 1:1. Sixty two percent of the subjects were infants. There were more acyanotic than cyanotic CHDs (80.6% vs 19.4%). The most common types of ventricular septal defect (VSD) and atrial septal defect (ASD) were the membranous and ostium secundum types respectively. The medium sized VSD (49.0%) were in the majority while the small size patent ductus arteriosus and atrial septal defects predominated, 69.5% and 61.3% respectively., Conclusion: The prevalence of CHD in this study was high. The large number of small sized defects of ASD and VSD allows for spontaneous closure or uncomplicated surgical repair of the defects, an advantage in a setting with poor access to heart surgery., Competing Interests: The Authors declare that no competing interest exists., (Copyright © 2022 by West African Journal of Medicine.)

Objective: The ostium secundum atrial septal defect (ASD) is among the most common congenital cardiac anomalies diagnosed in adulthood. A known complication of transcatheter ASD closure is the development of new-onset atrial fibrillation and flutter (AFi/AFl). These arrhythmias confer an increased risk of postoperative stroke, thrombus formation and systemic emboli. This systematic review examines the burden of de novo AFi/AFl in adults following transcatheter closure and seeks to identify risk factors for AFi/AFl development., Methods: Studies were identified by a search of MEDLINE, EMBASE and Cochrane databases from inception until 29 April 2020. A meta-analysis of AFi/AFl incidence was performed using a random-effects model., Results: A total of 31 studies met inclusion criteria, comprising 4788 adult patients without a history of AFi/AFl. Twenty-three studies were included in quantitative synthesis and demonstrated an overall incidence rate of 1.82 patients per 100 person-years of follow-up (I 2 =83%). In studies that enrolled only patients ≥60 years old, the incidence was 5.21 patients per 100 person-years (I 2 =0%). Studies with follow-up duration ≤2 years reported an incidence of 4.05 per 100 person-years (I 2 =55%) compared with a rate of 1.19 per 100 person-years (I 2 =85%) for studies with follow-up duration >2 years., Conclusions: The incidence of new-onset AFi/AFl is relatively low following transcatheter closure of secundum ASDs. The rate of de novo AFi/AFl, however, was significantly higher in elderly patients. Shorter follow-up time was associated with a higher reported incidence of AFi/AFl., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Objectives: The long-term clinical benefits of atrial septal defect (ASD) closure remain controversial. We aimed to compare long-term clinical outcomes between patients who underwent early surgical closure after ASD diagnosis and those who did not., Methods: Using the Korean National Health Insurance Service database, we identified patients with isolated ASDs diagnosed between 1 January 2003 and 31 December 2006. The ASD patients who underwent closure surgery within 6 months after diagnosis were allocated to the early-closure group and the rest were allocated to the conservative-strategy group. The primary outcome was all-cause mortality. Secondary outcomes were atrial flutter/fibrillation (AFF) and ischaemic stroke., Results: Among patients without a history of AFF or stroke, 1644 patients in the early-closure group were propensity score matched to 1644 patients in the conservative-strategy group and their median follow-up durations were 12.9 and 12.8 years, respectively. The early closure was associated with a significantly lower risk of mortality (hazard ratio, 0.55 [95% confidence interval, 0.43-0.70]). In an age-stratified analysis, significant mortality reductions in the early-closure group were found in patients aged 40 years or older. The risk of AFF was significantly higher in the early-closure group, which might be mainly ascribed to postoperative transient AFF, while there was no difference in ischaemic stroke between the 2 groups., Conclusions: Our data suggest that timely ASD closure without delay is necessary for ASD patients without previous history of clinical events, especially in patients aged 40 years or older., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Patients with atrial septal defects (ASDs) have increased mortality and morbidity. This can only partly be explained by hemodynamic changes caused by the ASD, suggesting additional underlying causes. Patients with an ASD have an increased burden of pathogenic gene variants in ASD-related genes, indicating genetics as an important factor in etiology. Inheritance of genetic variants with high impact can cause ASD in relatives (familial ASD). This study aimed to investigate whether lifelong outcomes were different in patients with familial ASD compared with patients with sporadic ASD. We used health registries and a nationwide cohort of 2,151 patients with ASD to compare the incidences of atrial fibrillation or flutter (together abbreviated as AF), heart failure, and mortality between patients with familial and sporadic ASD using Cox proportional hazard ratio and Fine and Gray analysis. Patients with familial ASD experienced AF and heart failure earlier in life than patients with sporadic ASD, with hazard ratios of 1.6 and 1.7, respectively. Subdistribution hazard ratios showed an increased risk of AF and heart failure in patients with familial ASD compared with patients with sporadic ASDs (2.3 and 3.1, respectively). Our results suggest that genetic variants with high impact may influence the outcomes of patients with ASD. In conclusion, patients with familial ASD have an increased risk and an earlier onset of AF and heart failure compared with patients with sporadic ASD, hence clinical awareness of arrhythmias and heart failure in patients with familial ASD may lead to timely treatment., Competing Interests: Disclosures The authors have no conflicts of interest to declare., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Introduction: Annually, 7.9 million neonates in the world have developmental anomalies. Together with prema turity, they constitute the main causes of mortality during the first year of life in developed and de veloping countries. In Chile, the estimated prevalence is 3.9% of all live births. There are no reports on the prevalence of malformations in the Araucanía Region., Objective: to estimate the prevalence of congenital malformations at birth in neonates hospitalized in a Neonatology Service., Patients and Method: cross-sectional study. Reference population: 54,241 live births at the Regional Hospi tal of Temuco over a 10-year period. Cases came from the neonatology hospital discharge database according to the ICD 10 classification, from January 1, 2009, to December 31, 2018. Descriptive and analytical statistics were performed with the STATA 15 software., Results: 949 neonates with one or more congenital malformations were identified. The overall prevalence of neonates with malforma tions was 1.7%, the most prevalent being ventricular septal defect 40.9 x 10,000 live newborns (LNB), atrial septal defect 21.5 x 10,000 LNB, cleft lip and palate 14.0 x 10,000 LNB, congenital hypertrophic cardiomyopathies 8.1 x 10,000 LNB, and congenital rectal atresia and stenosis or absence of the anus 7.9 x 10,000 LNB., Conclusions: the overall prevalence of malformations is similar to that reported for the country. When analyzing by type, we found significantly higher incidences than those repor ted in previous studies.

Purpose: The atrial septal pouch is an anatomic variant of the interatrial septum caused by incomplete fusion of the septum primum and secundum. It is debatable whether coexistence of septal pouch and atrial fibrillation (AF) increases the risk of stroke. Thus, the aim of the study was to evaluate the coexistence of left atrial septal pouch (LASP) and AF in patients with a history of stroke in comparison with those without an earlier history of stroke., Materials and Methods: We analyzed 145 patients with AF (68 in the study group, 77 in the control group). The study group consisted of patients with previous stroke and AF; control group consisted of patients scheduled for AF ablation. All patients underwent computed tomography with the assessment of atrial septum morphology and presence of LASP. The study was a subanalysis of the ASSAM (AssesSment of the left atrial appendage morphoLogy in patients aAfter ischaeMic Stroke) study., Results: The prevalence of LASP in the entire group was 38.6%. There were no significant differences in the prevalence of LASP between the study and control groups (33.8% vs. 42.9%, P=0.265). Mean longitudinal and transverse dimensions of LASP were 9.34±3.27 and 2.4±0.6 mm, respectively, and there was no significant difference between the study and controls. Patients from the stroke group were older (P<0.0001), had a higher CHA2DS2-VASc score (5.41±1.93 vs. 1.62±1.46, P<0.0001) compared with controls., Conclusions: Presence of LASP is not more prevalent in patients with a history of stroke. Additional information on the presence of LASP do not improve accuracy of risk stratification for stroke in patients with AF., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To investigate the rate and clinical impact of a persisting iatrogenic atrial septal defect (iASD) after percutaneous left atrial appendage occlusion (LAAO)., Background: Percutaneous LAAO is an alternative to oral anticoagulation (OAC) for the prevention of ischemic stroke and systemic embolism in patients with atrial fibrillation (AF). Data regarding incidence and persistence of iASD after LAAO procedures and its clinical relevance is scarce., Methods: We retrospectively analyzed 144 patients that underwent LAAO at our center between 2009 and 2020 who had at least one follow-up including transesophageal echocardiography (TEE). Baseline clinical, procedural data and echocardiographic characteristics in patients with and without evidence of an iASD were compared. We furthermore determined the rate of iASD persistence over time and evaluated outcomes of patients with and without spontaneous iASD closure., Results: After a median of 92 days (IQR 75-108 days) after LAAO, 50 patients (50/144, 34.7%) showed evidence of an iASD. Patients with iASD had higher CHADS-VASc-scores (4.9±1.5 vs 4.2±1.2, p = 0.03), larger left atrial volumes (80.5±30.5 ml vs 67.1±19.7 ml, p = 0.01) and were more likely to have relevant mitral regurgitation (≥° II) (46.0% vs 12.3%, p = 0.001). LAAO procedures took longer (50.1±24.3 vs 41.1±17.8 min, p = 0.06) in patients with a persisting iASD. Furthermore, larger device sizes were implanted (24.3±3.4 mm vs 22.1±2.8 mm, p = 0.03). The presence of an iASD had no impact on RV dysfunction, thromboembolism or mortality. Spontaneous closure of an iASD was documented in 52.0% (26/50). Hereby, similar risk factors were identified for the persistence of an iASD in follow-up., (© 2021 The Authors. Echocardiography published by Wiley Periodicals LLC.)

Objectives: Secundum atrial septal defect (ASD) is a common adult congenital heart lesion for which percutaneous closure offers treatment in patients with suitable anatomy. We sought to determine the outcomes of coronary angiography in a population of adults >40 years of age who were undergoing percutaneous ASD closure., Methods: Patients >40 years of age who underwent ASD closure between 2009-2016 were included in this retrospective chart review. Coronary angiograms were reviewed by 2 independent reviewers to evaluate the presence and degree of coronary artery disease (CAD) and the resulting clinical sequelae., Results: A total of 398 patients underwent ASD closure, and 300 had coronary angiography at the time of closure. Mild CAD (10%-39% vessel stenosis) was found in 33 patients (11.0%), moderate CAD (40%-69% vessel stenosis) in 23 patients (7.7%), and severe CAD (≥70% vessel stenosis) in 25 patients (8.3%). Of the 48 patients with moderate to severe CAD, 24 had proximal vessel disease and 21 had multivessel disease. Four patients (8.3%) with moderate to severe CAD subsequently underwent percutaneous interventions, 16 patients (33.3%) had medication changes, 16 patients (33.3%) had perfusion testing followed by medication changes, and 12 patients (25%) had no changes in their medical management., Conclusions: Given the prevalence of CAD in this population, routine coronary angiography at the time of ASD closure should be reserved for patients with an unfavorable cardiovascular risk profile, who have a higher likelihood of CAD. While patients with ASDs suffer from chest pain and dyspnea both before and after percutaneous closure, few have established CAD.

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

Background: New-onset atrial tachyarrhythmia (ATA) often develops after atrial septal defect (ASD) closure. Its development raises some potential concerns such as stroke and bleeding complications caused by anticoagulant therapy and limited access to the left atrium for catheter ablation. Although it is essential to identify the risk factors of new-onset ATA, few studies have examined these factors. This study investigated unknown risk factors for the development of new-onset ATA after transcatheter ASD closure in patients without a history of ATA., Methods: A total of 238 patients without a history of ATA, aged ≥18 years and who underwent transcatheter ASD closure at the current hospital were reviewed. Patient characteristics were compared between the groups with and without new-onset ATA. The factors associated with new-onset ATA were examined using univariate and multivariable analyses., Results: Thirteen (13) (5.5%) patients experienced ATA during follow-up (mean, 21±14 months). Compared with patients without new-onset ATA, patients with new-onset ATA were older (48±18 vs 66±11 years; p<0.001) and had high brain natriuretic peptide (BNP) levels (36±36 vs 177±306 pg/mL; p<0.001). On multivariable analysis, BNP ≥40 pg/mL before ASD closure was associated with new-onset ATA after adjusting for age (OR, 4.91; 95% CI, 1.22-19.8; p=0.025)., Conclusion: Patients with BNP levels >40 pg/mL before transcatheter ASD closure may have a higher risk of developing new-onset ATA., (Copyright © 2021 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Background: The association between maternal exposure to gaseous air pollutants and congenital heart defects (CHD) remains unclear. The concentration-response relationship and the time windows of susceptibility to gaseous pollutants may vary by pollutant species and CHD subtypes., Objective: We aimed to examine the relationship between maternal exposures to four species of gaseous pollutants (NO 2 , O 3 , SO 2 , and CO) and atrial septal defect (ASD), which is a common subtype of CHD, and to determine the critical time windows of susceptibility for each gaseous pollutant., Methods: Among 1,253,633 infants born between October 1, 2013 and December 31, 2016 in China, 1937 newborns were diagnosed with isolated ASD, a prevalence of 1.55‰. Maternal exposures to the gaseous pollutants were estimated by matching the geocoded maternal addresses with the gridded ambient concentrations. The adjusted odds ratios (aOR) between exposures and ASD were quantified by using mixed-effects logistic regression models., Results: We found significantly positive associations between ASD and maternal exposures to NO 2 , O 3 , SO 2 , and CO during entire pregnancy, first-, second-, and third-trimester. However, no statistically significant association was found between maternal exposure to PM 2.5 , PM 2.5-10 and ASD risk (P > 0.05). In the fully adjusted model with respect to average exposure over entire pregnancy, the adjusted odds ratios (aOR) for each 10 μg/m 3 increment of NO 2 , O 3 , SO 2 were 1.33 (95% CI: 1.22-1.45), 1.13 (95% CI: 1.10-1.16), 1.28 (95% CI: 1.20-1.35), respectively; the aOR for each 100 μg/m 3 increment of CO was 1.10 (95% CI: 1.06-1.15). The observed concentration-response relationships varied by exposure periods and pollutants, with the strongest association for NO 2 during the 1st-8th embryology weeks, for O 3 during the third trimester, for SO 2 during the second trimester, and for CO without obvious variation., Conclusions: The findings suggest an increased risk of ASD in association with maternal exposures to four common gaseous pollutants. From the perspective of birth defects prevention and ASD risk mitigation, it is critical to reduce maternal exposure to gaseous pollutants especially during the most susceptible time windows., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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

With an increase in number of patients undergoing percutaneous treatment of severe mitral regurgitation with MitraClip placement, the consequences of transseptal puncture resulting in iatrogenic atrial septal defects (iASDs) are being increasingly realized. Closure of iASDs following MitraClip therapy is not routinely performed, and no guidelines currently exist in managing this condition. While immediate hemodynamic and clinical compromise secondary to acute hypoxemia related to iASDs should be acutely managed with defect closure, the prevalence and consequences of long-term iASDs are still unclear. Some studies have cited a potential improvement in hemodynamic outcomes as a result of iASDs; while others report potential inferior and even fatal outcomes. In this state-of-the-art clinical review, we present the readers with the current data on the prevalence, outcomes, and potential management options of iASDs after MitraClip placement., (© 2020 Wiley Periodicals LLC.)

Background: Shape-memory abnormalities are seen in some nitinol atrial septal occluders. Variably described as cobra-head, tulip, and others, their incidence, mechanisms, clinical impact, and outcome have not been systematically analysed., Methods: We retrospectively reviewed all consecutive device closures in the last 6 years for deformations. Type and size of the occluder, deployment technique, size, and angulation/kinking of the delivery sheath were analysed. Procedural success, duration, and other complications were studied., Results: A total of 112 devices (11.8%) among 950 occluders used in 936 patients showed deformities. Fourteen of 936 received 2 devices. Deformities were transient and self-correcting in 40%. Multivariate analysis showed significant associations with oversized sheaths (p = 0.004), kinked/angulated sheaths (p < 0.001), special deployment techniques (p < 0.001), and twist in the device waist (p = 0.011). Despite more frequent deformities with Figulla (15.6%) and Amplatzer (13.9%) occluders than Cera occluders (6.6%) and larger devices (>24 mm - 14.6%) than smaller devices (less than or equal to 24 mm - 9.7%), they were not significant on multivariate analysis. In vivo manipulations corrected most deformities; nineteen needed in vitro reformations and four needed a change of device. Despite prolongation of the procedure, repeated attempts (mean 2.76 ± 1.7 attempts, with a range from 1 to 9 attempts), and supraventricular tachycardia in two patients, there were no serious adverse effects., Conclusions: Deformations were frequent in 11.8% of atrial septal occluders on a targeted search. Oversized and angulated/kinked sheaths, special techniques like pulmonary vein deployment and twist in device waist during procedure predisposed to deformities. While most deformities were corrected with manipulations, removal of the device was infrequently needed and change of device was rarely required. Long procedural time and multiple attempts for deployment did not affect procedural success.

Background: An isolated coronary sinus (CS) atrial septal defect (ASD) is defined as a CS unroofed in the terminal portion without a persistent left superior vena cava or other anomalies. This defect is rare and part of the wide spectrum of unroofed CS syndrome (URCS). Recently, several reports have described this finding. The database of New Tokyo Hospital was searched to determine the incidence of this defect. Additionally, to raise awareness of this condition, the findings from five patients with CS ASD who underwent surgical repair at New Tokyo Hospital are discussed., Case Presentation: The patients were three women and two men with an age range of 63-77 years. All patients underwent transthoracic echocardiography and computed tomography, and one underwent magnetic resonance imaging. In two patients, the defect was found unexpectedly intraoperatively; left-to-right shunting was apparent in the other three patients preoperatively. The pulmonary-to-systemic blood flow ratio ranged from 1.42 to 3.1 following cardiac catheterization, and oxygen saturation step-up was seen on the right side of the heart. Valvular regurgitation was seen in 4/5 patients with different combinations and degrees of mitral, tricuspid, and aortic valve involvement. Right atrial and ventricular dilation were seen in 4/5 patients; three patients had left atrial dilation. Three patients experienced atrial fibrillation, and one of these also experienced paroxysmal ventricular contractions. All patients underwent surgical repair, and some underwent multiple procedures. One patient who had previously undergone kidney transplantation died approximately 1 year postoperatively; the remaining four patients are currently experiencing good activities of daily living without symptoms., Conclusions: CS ASD (Kirklin and Barratt-Boyes type IV URCS) comprised 1.3% of adult congenital heart surgeries and 0.07% of adult open-heart surgeries at New Tokyo Hospital from 1999 to 2019. At New Tokyo Hospital, cardiac surgery is performed mainly for patients with acquired cardiac disease, and CS ASD is rare. Early diagnosis is important, as well as early surgical repair in symptomatic patients, especially those with blood access shunts, which may overload the heart. The case of a poor prognosis in this series is noteworthy, as similar cases have not been reported previously.

Introduction: Congenital heart defects (CHDs) are the most common congenital defects and accounts for nearly one-third of all major congenital anomalies. It is the leading causes of birth defect-associated morbidity, mortality, and medical expenditures. Of all CHD types, ventricular septal defect (VSD) and atrial septal defect (ASD) accounted 51% of cases with an increasing trend over time., Objective: The aim of this review is to estimate the pooled prevalence of ventricular septal defect and congenital atrial septal defect among congenital heart diseases patients in East African context., Methods: Using PRISMA guideline, we systematically reviewed and meta-analyzed studies that examined the prevalence of Ventricular septal defect and atrial septal defect in East Africa, from Medline (PubMed), Cochrane Library, HINARI, and Google Scholar. A weighted inverse variance random-effects model was used to estimate the pooled prevalence of ventricular septal defect and atrial septal defect., Results: A total of 2323 studies were identified; 1301 from PubMed, 12 from Cochrane Library, 1010 from Google Scholar and 22 from other sources. The pooled prevalence of ventricular septal defect and atrial septal defect in East Africa was found to be 29.92% (95% CI; 26.12-33.72; I2 = 89.2%; p<0.001), and 10.36% (95% CI; 8.05-12.68; I2 = 89.5%; p<0.001) respectively., Conclusions and Future Implications: Based on this review, the pooled prevalence of VSD and ASD is still high and alarming; this signifies that the emphasis given for congenital heart defect in East African countries is limited. Special attention and efforts should be applied for early detection to prevent serious complications and for a better prognosis of all forms of CHD. A screening program for CHD should be instituted during the perinatal period. Furthermore, early referral of suspected cases of congenital cardiac anomalies is mandatory for better management till the establishment of cardiac centers in different regions of the continent., Competing Interests: The authors declare that they have no competing interests.

The left atrial septal pouch (LASP) occurs due to incomplete fusion of septa primum and secundum at the inter-atrial septum, creating an open flap that may serve as a thromboembolic source. Prior studies have demonstrated increased prevalence of LASP in cryptogenic strokes. The aim of the current study was to validate the above findings in a separate, larger group of stroke and non-stroke patients. We examined transesophageal echocardiograms (TEEs) performed between July 2011 and December 2018. LASP prevalence was determined in TEEs referred for ischemic stroke or transient ischemic attack ("stroke") and compared with LASP prevalence in patients undergoing TEEs for other reasons ("non-stroke"). Stroke subtyping was performed using the Trial of Org 10172 in Acute Stroke Treatment (TOAST) criteria. There were 306 TEEs from 144 non-stroke and 162 stroke patients. Mean age and sex distribution were 56 ± 1 (mean ± SE) and 65% male in the non-stroke group and 58 ± 1 and 54% male in the stroke group. The overall prevalence of LASP was 31%. The prevalence of LASP was 28% (41/144) in non-stroke patients, 25% (24/95) in non-cryptogenic stroke patients, and 43% (29/67) in cryptogenic stroke patients. LASP prevalence was significantly higher in the cryptogenic subgroup compared with the non-cryptogenic subgroup (p = 0.02). These findings demonstrate a significant association of LASP with risk of cryptogenic stroke, suggesting that LASP may serve as a thromboembolic nidus. Additional studies are needed to determine the generalizability of these findings, and their therapeutic implications, supporting LASP as a stroke risk factor.

Introduction: Technologically, advances in both transcatheter and surgical techniques have been continuing in the past 20 years, but an updated comprehensive comparison in device-based versus surgery in adults in terms of incidence of in-hospital mortality, perioperative stroke, and atrial fibrillation onset is still lacking. We investigate the performance of transcatheter device-based closure compared to surgical techniques by a systematic review and meta-analysis of the last 20 years literature data., Material and Methods: The analysis was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Literature search was performed based on Cochrane Library, Embase, PubMed, and Google Scholar to locate articles published between January 2000 and October 2020, regarding the comparison between short-term outcome and post-procedural complications of atrial septal defect patients receiving transcatheter or surgical closure. The primary outcome was the comparison of in-hospital mortality from all causes between patients treated with transcatheter closure or cardiac. Secondary outcomes were the onset of post-procedural atrial fibrillation or perioperative stroke., Results: A total of 2360 patients were included of which 1393 [mean age 47.6 years, 952 females (68.3%)] and 967 [mean age 40.3 years, 693 females (71.6%)] received a transcatheter device-based and surgery closure, respectively. In-hospital mortality [OR 0.16 (95% CI (0.66-0.44)), p = 0.0003, I2 = 0%], perioperative stroke [OR 0.51 (95% CI (0.31-0.84)), p = 0.003, I2 = 79%], and post-procedural atrial fibrillation [OR 0.14 (95% CI (0.03-0.61)), p = 0.009, I2 = 0%] significantly favoured transcatheter device-based closure., Conclusion: Transcatheter atrial septal defect closure resulted safer in terms of in-hospital mortality, perioperative stroke, and post-procedural atrial fibrillation compared to traditional surgery.

Importance: In early 2020, the United States declared a public health emergency in response to coronavirus disease 2019 (COVID-19) and implemented a variety of social distancing measures. The association between the COVID-19 pandemic and the number of pediatric admissions is unclear., Objective: To determine the changes in patterns of pediatric admissions in 2020 compared with the prior decade., Design, Setting, and Participants: This cross-sectional study included 49 US hospitals contributing to the Pediatric Health Information Systems database. Inpatient admissions were transformed into time-series data, and ensemble forecasting models were generated to analyze admissions across a range of diagnoses in 2020 compared with previous years. The setting was inpatient admissions. All patients discharged between January 1, 2010, and June 30, 2020, from an inpatient hospital encounter were included., Main Outcomes and Measures: Number of hospital admissions by primary diagnosis for each encounter., Results: Of 5 424 688 inpatient encounters among 3 372 839 patients (median [interquartile range] age, 5.1 [0.7-13.3] years; 2 823 748 [52.1%] boys; 3 171 224 [58.5%] White individuals) at 49 hospitals, 213 571 (3.9%) were between January 1, 2020, and June 30, 2020. There was a decrease in the number of admissions beginning in March 2020 compared with the period from 2010 to 2019. At the nadir, admissions in April 2020 were reduced 45.4% compared with prior years (23 798 in April 2020 compared with a median [interquartile range] of 43 550 [42 110-43 946] in April 2010-2019). Inflation-adjusted hospital charges decreased 27.7% in the second quarter of 2020 compared with prior years ($4 327 580 511 in 2020 compared with a median [interquartile range] of $5 983 142 102 [$5 762 690 022-$6 324 978 456] in 2010-2019). Seasonal patterns were evident between 2010 and 2019 for a variety of common pediatric conditions, including asthma, atrial septal defects, bronchiolitis, diabetic ketoacidosis, Kawasaki syndrome, mental health admissions, and trauma. Ensemble models were able to discern seasonal patterns in admission diagnoses and accurately predicted admission rates from July 2019 until December 2019 but not from January 2020 to June 2020. All diagnoses except for birth decreased below the model 95% CIs between January 2020 and June 2020., Conclusions and Relevance: In this cross-sectional study, pediatric admissions to US hospitals decreased in 2020 across an array of pediatric conditions. Although some conditions may have decreased in incidence, others may represent unmet needs in pediatric care during the COVID-19 pandemic.

Background: Atrial arrhythmias are common in patients with atrial septal defects (ASD) but the effects of percutaneous closure on atrial arrhythmia prevalence is unclear. We investigated the effects of ASD device closure and the impact of age at time of closure on prevalent atrial arrythmia., Methods: Meta-analysis of studies reporting atrial arrhythmia prevalence in adult patients before and after percutaneous closure was performed. Primary outcomes were prevalence of 'all atrial arrhythmia' and atrial fibrillation alone post closure. Sub-group analysis examined the effects of closure according to age in patients; <40 years, ≥40 and ≥ 60 years. 25 studies were included., Results: Meta-analysis of all studies demonstrated no reduction in all atrial arrhythmia or atrial fibrillation prevalence post-closure (OR 0.855, 95% CI 0.672 to 1.087, P = .201 and OR 0.818, 95% CI 0.645 to 1.038, P = .099, respectively). A weak reduction in all atrial arrhythmia and atrial fibrillation was seen in patients ≥40 years (OR 0.77, 95% CI 0.616 to 0.979, P = .032 and OR 0.760, 95% CI 0.6 to 0.964, P = .024, respectively) but not ≥60 years (OR 0.822, 95% CI 0.593 to 1.141, P = .242 and OR 0.83, 95% CI 0.598 to 1.152, P = .266, respectively). No data were available in patients <40 years. This, and other limitations, prevents conclusive assessment of the effect of age on arrhythmia prevalence., Conclusions: Overall, percutaneous ASD closure is not associated with a reduction in atrial arrhythmia prevalence in this meta-analysis. A weak benefit is seen in patients ≥40 years of age, not present in patients ≥60 years., Competing Interests: Declaration of Competing Interest Professor Mark O'Neill has received research support and honoraria from Biosense Webster and St. Jude Medical. Dr. Steven Williams has received research support from Biosense Webster. The remaining authors have nothing to disclose., (Copyright © 2020. Published by Elsevier B.V.)

Background: Atrial septal defect (ASD) is a common form of congenital heart disease. Significant shunts may increase the risk of developing pulmonary hypertension (PH). We aimed to describe current PH definitions, evaluate PH prevalence and the effect of PH on outcomes in patients undergoing percutaneous ASD closure., Methods: EMBASE, MEDLINE and Cochrane databases were systematically searched. Studies reporting PH prevalence or mean systolic pulmonary arterial pressure (sPAP) before and after percutaneous ASD closure in adults were included. We conducted meta-analyses to obtain summary estimates for PH prevalence and mean sPAP., Results: 15 articles with a total of 1073 patients met the eligibility criteria. Studies applied variable PH definitions. PH prevalence and mean sPAP levels decreased in all studies after closure. The pooled PH prevalence decreased from 44% (95% CI 29-60%) to 18% (95% CI 8-27%). The overall standardised mean difference in sPAP was 1.12 (95% CI 0.81-1.44) and 1.62 (95% CI 1.00-2.23) in cohort and case-series studies respectively indicating a large decrease. The pooled standard mean difference among the younger and older patients were different, 1.25 (95% CI 0.78-1.71) and 0.91 (95% CI 0.56-1.27), respectively. A high degree of between-study heterogeneity was noted., Conclusions: Both PH prevalence and mean sPAP decrease after ASD closure. Larger, prospective studies with consistent PH definitions using the recommended measurement modality are warranted., Competing Interests: Conflict of interest: S. Akseer has nothing to disclose. Conflict of interest: E. Horlick has nothing to disclose. Conflict of interest: V. Vishwanath has nothing to disclose. Conflict of interest: B. Hobbes has nothing to disclose. Conflict of interest: E. Huszti has nothing to disclose. Conflict of interest: S. Mak has nothing to disclose. Conflict of interest: D. Lee has nothing to disclose. Conflict of interest: L. Abrahamyan has nothing to disclose., (Copyright ©ERS 2020.)

Background: The purpose of this study was to prospectively recruit patients treated with limb malformation and to explore the prevalence and the clinical and epidemiological features of Heart-Hand Syndrome (HHS) in China., Methods: The consecutive patients treated for congenital upper limb malformation in Beijing Ji Shui Tan Hospital from October 1st, 2016 to October 1st, 2019 were prospectively recruited. We reviewed the patients' medical records and identified patients with abnormal electrocardiogram (ECG) and/or abnormal ultrasonic cardiogram as well as their basic demographic and clinical characteristics., Results: A total 1653 (1053 male and 600 female) patients with congenital upper extremity malformations were prospectively recruited. Among them, 200 (12.1%) had abnormal ultrasonic cardiogram (181patients, 10.9%) and/or abnormal ECG (19 patients, 1.1%). The commonest type of abnormal heart structure was atrial septal defect (69/181 38.1%), and the commonest abnormal ECG was wave patterns (7/19, 36.8%). HHS patients had a higher comorbidity rate (11%) than non-HHS patients (6.9%). Patients with HHS were classified into four groups by the types of congenital upper extremity malformations, among which the most common group was thumb type (121/200, 60.5%)., Conclusions: HHS occurred frequently among patients with congenital upper extremity malformation in China, particularly for those with multiple congenital malformations. The commonest type of hand malformations of HHS patients was thumb malformation.

Objective: Currently, consensus is lacking on the relation between closure of atrial septal defect (ASD) and the incidence of atrial fibrillation (AF), which is a known complication in ASD patients. More importantly, studies reporting on the treatment applied for AF in ASD patients are scarce. The aims of this study were (1) to assess the incidence of AF in ASD patients, (2) to study the relation between closure and AF and (3) to evaluate applied treatment strategies., Methods: A single-centre retrospective study in 173 patients with an ASD was performed. We analysed the incidence of AF, the relation of AF with closure, method of closure and the treatment success of therapies applied., Results: Almost 20% of patients with an ASD developed AF, with a mean age of 59 (±14) years at first presentation of AF during a median clinical follow-up of 43 (29-59) years. Older age (OR 1.072; p<0.001) and a dilated left atrium (OR 3.727; p=0.009) were independently associated with new-onset AF. Closure itself was not independently associated with AF. First applied treatment strategy was rhythm control in 77%. Of the 18 patients treated with antiarrhythmic drugs 50% had at least 1 recurrence of AF., Conclusion: No clear relation between closure of the ASD and AF could be assessed. This is the first study describing applied therapy for AF in ASD patients of which medical rhythm control was the most applied strategy with a disappointing efficacy., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

In this nation-wide cohort study we report the first long-term results of the association between having a atrial septal defects (ASD) on psychiatric disorders and use of psychotropic agents. Through population-based registries we included Danish individuals born before 1994 who received an ASD diagnosis between 1959and 2013. We used Cox proportional hazards regression and Fine and Grey competing risk regression to estimate the risk of receiving a psychiatric diagnosis and use of psychotropic medicine compared with a gender and age matched background population cohort. In 2,277 patients with a median follow-up from ASD diagnosis of 23.4 years (range 0.2 to 59.3 years) we found ASD patients to have a higher risk of psychiatric disorders (adjusted hazard ratio [HR]: 3.9; 95% confidence interval [CI] 3.4 to 4.5) compared with the comparison cohort and a cumulative incidence of using psychotropic agents 30 years after the ASD diagnosis of 47.4% (95% CI: 40.3 to 55.1) in the ASD patients and 25.5%, (95% CI: 23.5 to 27.8) in the comparison cohort. Diagnosis of the ASD before the age of 15 years (adjusted HR: 3.4; 95% confidence interval: 2.0 to 4.0) and surgical correction of the defect (HR: 1.5 (95% CI: 1.2 to 1.8), p <0.0001) had a higher risk than those with an ASD diagnosis after the age of 15 years and those with transcatheter closure of the defect. In conclusion, ASD patients had increased long-term risk of psychiatric disorder and use of psychotropic agents compared with a gender and age matched general population controls., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The epidemiology of Congenital Heart Diseases (CHDs) has changed over the past twenty years. This study aimed to evaluate the prevalence of CHDs in the population of dogs recruited in a single referral center (RC); compare the epidemiological features of CHDs in screened breeds (Boxers) versus non-screened (French and English Bulldogs, German Shepherds); investigate the association of breeds with the prevalence of CHDs; determine the popularity and volatility of breeds over a 20-year period; analysed the trends of the most popular breeds in the overall population of new-born dogs registered in the Italian Kennel Club (IKC) from 1st January 1997 to 31st December 2017. The RC's cardiological database was analysed, and 1,779 clinical records were included in a retrospective observation study. Descriptive statistics and frequencies regarding the most representative breeds and CHDs were generated. A logistic regression model was used to analyse the trends of the most common CHDs found in single and in cluster of breeds. The relationship between breed popularity and presence of CHDs was studied. The most common CHDs were Pulmonic Stenosis, Patent Ductus Arteriosus, Subaortic Stenosis, Ventricular Septal Defect, Aortic Stenosis, Tricuspid Dysplasia, Atrial Septal Defect, Double Chamber Right Ventricle, Mitral Dysplasia, and others less frequent. The most represented pure breeds were Boxer, German Shepherd, French Bulldog, English Bulldog, Maltese, Newfoundland, Rottweiler, Golden Retriever, Chihuahua, and others in lower percentage. Chihuahuas, American Staffordshire Terriers, Border Collies, French Bulldogs, and Cavalier King Charles Spaniel were the most appreciated all of which showed a high value of volatility. This study found evidence for the value of the screening program implemented in Boxers; fashions and trends influence dog owners' choices more than the worries of health problems in a breed. Effective breeding programs are needed in order to control the diffusion of CHDs without impoverishing the genetic pool., Competing Interests: The authors have declared that no competing interests exist.

Objective: To determine the prevalence of pacemaker and conduction disturbances in patients with atrial septal defects., Design: All patients with an atrial septal defect born before 1994 were identified in the Danish National Patient Registry, and 297 patients were analysed for atrioventricular block, bradycardia, right bundle branch block, left anterior fascicular block, left posterior fascicular block, pacemaker, and mortality. Our results were compared with pre-existing data from a healthy background population. Further, outcomes were compared between patients with open atrial septal defects and atrial septal defects closed by surgery or transcatheter., Results: Most frequent findings were incomplete right bundle branch block (40.1%), left anterior fascicular block (3.7%), atrioventricular block (3.7%), and pacemaker (3.7%). Average age at pacemaker implantation was 32 years. Patients with defects closed surgically or by transcatheter had an increased prevalence of atrioventricular block (p < 0.01), incomplete right bundle branch block (p < 0.01), and left anterior fascicular block (p = 0.02) when compared to patients with unclosed atrial septal defects. At age above 25 years, there was a considerably higher prevalence of atrioventricular block (9.4% versus 0.1%) and complete right bundle branch block (1.9% versus 0.4%) when compared to the background cohorts., Conclusions: Patients with atrial septal defects have a considerably higher prevalence of conduction abnormalities when compared to the background population. Patients with surgically or transcatheter closed atrial septal defects demonstrated a higher demand for pacemaker and a higher prevalence of atrioventricular block, incomplete right bundle branch block, and left anterior fascicular block when compared to patients with unclosed atrial septal defects.

PURPOSE The increase in volume and pressure in the right atrium (RA) and right ventricle (RV) has been shown to increase the liver stiffness (LS). In the literature, there is no information about the changes in LS value in patients with atrial septal defect (ASD). The aim of our study was to investigate the change of LS values obtained by point shear-wave elastography (pSWE) in patients with ASD and the clinical utility of pSWE for this disease. METHODS This cross-sectional study included 66 patients with ostium secundum ASD: 21 patients with no indication for ASD closure (Group I), 38 patients who underwent ASD closure (Group II), and 7 patients who had ASD with Eisenmenger syndrome (Group III). All patients underwent echocardiography and pSWE. Increased LS was accepted as ≥7 kPa. RESULTS LS values as well as transaminases, clinical signs of heart failure and functional and structural heart abnormalities (increase of RA and RV diastolic dimensions, tricuspid regurgitation pressure gradient [TRPG], ASD size and decrease of ejection fraction, tricuspid annular plane systolic excursion) significantly increased from Group I to Group III (P < 0.001 for all comparisons). Mean LS values for Group I, Group II, and Group III were 5.16±1.55 kPa, 7.48±1.99 kPa, and 13.9±2.58 kPa, respectively. In multivariate linear regression analysis, ASD size and TRPG were significantly associated with LS increase. Only ASD size independently predicted abnormal LS increase ≥7 kPa according to multivariate logistic regression. Clinical value of LS increase was comparable to TRPG for detection of Eisenmenger syndrome; in the receiver operating curve analysis, area under the curve was 0.995 for LS (P < 0.001) and 0.990 for TRPG (P < 0.001). At 10 kPa threshold, LS determined the Eisenmenger syndrome with 100% sensitivity and 91.5% specificity. CONCLUSION LS value assessed by pSWE was significantly increased in ASD patients with closure indication and Eisenmenger syndrome compared to patients without ASD closure indication and was comparable with TRPG in regards to Eisenmenger syndrome identification. ASD size significantly associated with LS and independently predicted abnormal LS increase ≥7 kPa.

Background: Patients 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 Results: We 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., Conclusions: Based 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., Competing Interests: Declaration of competing interest The authors and the institutions involved have received support by Actelion, Pfizer and GlaxoSmithKline Group of Companies (GSK)., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Congenital heart disease (CHD) is the most common congenital anomaly in children. Over half of the deaths due to CHD occur in the neonatal period. Most children with unrepaired complex heart lesions do not live to celebrate their first birthday. We describe the spectrum of congenital heart disease in Uganda., Methods: We retrospectively reviewed the data of children with CHD who presented to the Uganda Heart Institute (UHI), Mulago Hospital Complex from 2007 to 2014., Results: A total of 4621 children were seen at the UHI during the study period. Of these, 3526 (76.3%) had CHD; 1941(55%) were females. Isolated ventricular septal defect (VSD) was the most common CHD seen in 923 (27.2%) children followed by Patent ductus arteriosus (PDA) 760 (22%) and atrial septal defects (ASD) 332 (9.4%). Tetralogy of Fallot (TOF) and Truncus arteriosus were the most common cyanotic heart defects (7% and 5% respectively). Dysmorphic features were diagnosed in 185 children, of which 61 underwent genetic testing (Down syndrome=24, 22q11.2 deletion syndrome n=10). Children with confirmed 22q11.2 deletion had conotruncal abnormalities., Conclusion: Isolated VSD and Tetralogy of Fallot are the most common acyanotic and cyanotic congenital heart defects. We report an unusually high occurrence of Truncus arteriosus., (© 2020 Namuyonga J et al.)

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

Objective: To study the incidence, types, and characteristics of CHD in all live births delivered in Jinshan Hospital from 1 January, 2016 to 31 December, 2017, and to analyse the correlation between CHD and the distance between maternal residence and Shanghai Petrochemical Complex., Methods: All live births, delivered in Jinshan Hospital in 2016 and 2017, have received CHD screening and neonatal follow-up after delivery. For those both positive for CHD screening and negative for CHD screening but with heart murmur found during physical examination on neonatal follow-up after delivery, echocardiography was performed to confirm CHD diagnosis. All maternal residential addresses have been grouped for analysis according to the distance between maternal residence and Shanghai Petrochemical Complex., Results: There were 5544 live births in total, and a total of 79 children with CHD were confirmed by CHD screening and echocardiography, of which the types of diseases with high incidence, in descending order of incidence, are atrial septal defect (48/79), ventricular septal defect (25/79), patent ductus arteriosus (21/79), and pulmonary artery stenosis (9/79). There was no statistically significant difference in the incidence of CHD among the groups divided by the distance between maternal residence and Shanghai Petrochemical Complex. The incidence of neonatal CHD near Shanghai Petrochemical Complex is 1.42%., Conclusions: Most of the children with CHD can be screened out through CHD screening and physical examination in neonatal period and early infancy stage. The distance between maternal residence and Shanghai Petrochemical Complex has no significant direct effect on the incidence of CHD in neonates near Shanghai Petrochemical Complex.

Introduction: Congenital heart defects (CHDs) are the most prevalent and severe type of major congenital anomalies (CAs). The objective of this study was to determine the frequency and distribution of CHDs in the Valencian Region from 2007 to 2014, describing common characteristics of the patients and their mothers., Material and Methods: We retrieved data on CHDs in live births, stillbirths and cases of termination of pregnancy for fetal anomaly between 2007 and 2014 (codes Q20-Q26 in the 10th Revision of the International Classification of Diseases-British Paediatric Association, ICD10-BPA) from the population-based Registry of congenital anomalies of the Valencian Region. We calculated the prevalence per every 10,000 births of CHDs overall and by subtype, analysed temporal trends and the geographic distribution of cases, and documented the presence of associated noncardiac malformations., Results: We identified 3,671 cases of CHD, corresponding to 38.6% of all CAs. The prevalence was 91.1/10,000 (IC 95%: 88.1-94.0) with a predominance of septal defects, chiefly atrial septal defect (48.5/10,000; IC 95%: 46.4-50.6) and ventricular septal defect (36.1/10,000; IC 95%: 34.3-38.0). We found the highest prevalence in the province of Castellon (137.8/10,000; IC 95%: 127.5-148.1). The sex distribution was 47.3% male and 44.3% female. Of all cases, 90.9% corresponded to live births and 65.6% were diagnosed at birth. The most frequent associated extracardiac malformations were musculoskeletal, and 19.2% of patients had syndromes. The most frequent maternal diseases were diabetes mellitus, hypothyroidism and urinary tract infections., Conclusions: The prevalence of CHD and atrial septal defects was higher compared to European data, while the prevalence of ventricular septal defects was similar. Musculoskeletal malformations were the noncardiac CAs most frequently associated with CHDs., (Copyright © 2019 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.)