Comparison of atrial septal defect and patent foramen ovale in cryptogenic strokes.

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.

Epidemiological Survey of Congenital Heart Disease Among Children Aged from 2 to 18 in Suo County, Nagqu, Tibet.

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.

Risk factors for isolated atrial septal defect secundum morbidity.

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.

Left Atrial Septal Pouch (LASP) and cryptogenic stroke risk: An updated systematic review and meta-analysis of observational studies.

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.

Prevalence of atrial septal defects and patent foramen ovale in a cohort of sudden cardiac death patients undergoing autopsy.

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.

Elevated risk for developmental disabilities in children with congenital heart defects.

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.

Outcomes of Percutaneous Atrial Septal Defect Closure With Mitral Transcatheter Edge-to-Edge Repair and Transseptal Mitral Valve Replacement (2015 to 2020).

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.

Atrial septal defect patients have an elevated risk for infective endocarditis.

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.

Maternal diabetes as a teratogenic factor for congenital heart defects in infants of diabetic mothers.

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.

Outcome of transcatheter atrial septal defect closure in a nationwide cohort.

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.

Cytogenetic study of subtypes of Down syndrome and its relation with pattern of congenital cardiac defects.

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.

Chiari network for the interventional cardiologist: A hidden enemy at the heart gate - A systematic review of the literature.

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.

Evaluation of the Prevalence and Anatomic Types of Congenital Heart Diseases: An Echocardiographic Study in a Tertiary Hospital in Nigeria.

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.

CONTEXTE: Les cardiopathies congénitales sont les anomalies congénitales les plus importantes. Le fardeau et les types sont importants pour la planification de la santé et la préparation à la chirurgie. Cette étude a été menée pour déterminer la prévalence, les types anatomiques et la taille des anomalies de la coronaropathie observés dans un hôpital tertiaire. MÉTHODES: Une revue rétrospective des dossiers échocardiographiques des enfants vus dans un centre tertiaire sur une période de douze ans. période. En utilisant les informations des échocardiogrammes, les types anatomiques et les tailles des défauts des maladies coronariennes ont été déterminés à l'aide de classifications connues. L'analyse statistique a été réalisée à l'aide d'IBM-SPSS version 21. RÉSULTATS: La prévalence de la coronaropathie était de 10,3/1000 enfants avec un rapport M:F de 1:1. Soixante-deux pour cent des sujets étaient des nourrissons. Il y avait plus de coronaropathies acyanotiques que cyanotiques (80,6 % vs. 19.4%). Les types les plus courants de communication interventriculaire (VSD) et de communication interauriculaire (ASD) étaient les types membraneux et ostium secundum respectivement. Les VSD de taille moyenne (49,0%) étaient majoritaires, tandis que le canal artériel persistant et la communication interauriculaire de petite taille étaient les plus fréquents. artérielle et les communications interauriculaires de petite taille prédominaient, respectivement 69,5 % et 61,3 %. 61,3 % respectivement. CONCLUSION: La prévalence des maladies coronariennes dans cette étude était élevée. Le grand nombre de défauts de petite taille de l'ASD et VSD permet une fermeture spontanée ou une fermeture sans complication. permet une fermeture spontanée ou une réparation chirurgicale non compliquée des chirurgicale sans complication, ce qui constitue un avantage dans un contexte où l'accès à la chirurgie cardiaque. Mots clés: Cardiopathie congénitale, types anatomiques, taille des défauts, échocardiographie. taille, échocardiographie.

Embolic Events After Computed Tomography Contrast Injection in Patients With Interatrial Shunts: A Cohort Study.

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.

[Prevalence of congenital malformations in newborns in the Araucanía region]. / Prevalencia de malformaciones congénitas en recién nacidos de la región de la Araucanía.

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.

Comparison of Outcome in Patients With Familial Versus Spontaneous Atrial Septal Defect.

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.

Clinical outcomes of early closure versus conservative strategy after diagnosis with atrial septal defect: a nationwide population-based cohort study.

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.

Left Atrial Septal Pouch-Is it Really a New Risk Factor for Ischemic Stroke?: Subanalysis of the ASSAM Study.

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.

Study of atrial arrhythmias after surgical or device closure of atrial septal defect.

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.