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

Background: Adults with simple congenital heart defects (CHD) have increased risk of neurodevelopmental challenges including executive dysfunction. It is unknown if the executive dysfunction is universal or if it is driven by dysfunction in specific clinical subscales and how it might affect psychosocial aspects of everyday life., Methods: The self-reported and informant-reported executive function of adults with an average age of 26 ± 5 (range 18-41) who underwent childhood surgery for atrial septal defects (n = 34) or ventricular septal defects (n = 32) and matched controls (n = 40) were evaluated using the Behavior Rating Inventory of Executive Functions - Adult version (BRIEF-A)., Results: The CHD group reported having more executive dysfunction than controls in all BRIEF-A clinical subscales (p < 0.020) and more than their informants reported on their behalf (p < 0.006). The CHD group had received three times more special teaching (44% compared to 16%) and pedagogical psychological counselling (14% compared to none) and had a three times higher occurrence of psychiatric disorders than controls (33% compared to 11%). Lower educational levels and psychiatric disorders were associated with higher BRIEF-A scores (p < 0.03)., Conclusions: Adults operated for septal defects in childhood report more challenges with all aspects of the executive functions than controls and more than relatives are aware of.

Objectives: Recent evidence suggests that patients with a corrected atrial septal defect (ASD) have higher morbidity and mortality. An abnormal autonomic regulation of the heart may be a part of the explanation for this. Our objective was to study heart rate variability (HRV) in adults with a corrected ASD as a prominent tool to investigate the autonomic regulation of the heart., Methods: Autonomic cardiac function was investigated in adults with either a surgically closed or percutaneously closed ASD and healthy control subjects. A 48-h Holter monitor was performed on each participant and HRV was assessed., Results: A total of 17 patients with surgically closed ASDs, 18 percutaneously closed ASDs and 18 controls were included. The mean age in the surgical group, percutaneous group and controls was 32 ± 9, 28 ± 7 and 32 ± 10 years, respectively. The mean time since closure was 19 ± 8 years for the surgical group and 15 ± 5 years for the percutaneous group. The surgically closed ASD patients showed decreased HRV in all six parameters studied when compared to the controls. Similarly, the percutaneously closed ASDs showed decreased HRV in three out of six parameters when compared to controls., Conclusions: Adults with an ASD, whether closed surgically or percutaneously, have impaired HRV compared to their age- and sex-matched controls, more so in the patients with a surgically closed ASD., Clinical Trial Registration Number: ClinicalTrials.gov (identifier: NCT03565471)., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Background: Many children born with congenital heart defects are faced with cognitive deficits and psychological challenges later in life. The mechanisms behind are suggested to be multifactorial and are explained as an interplay between innate and modifiable risk factors. The aim was to assess whether there is a relationship between mean arterial pressure during surgery of a septal defect in infancy or early childhood and intelligence quotient scores in adulthood., Methods: In a retrospective study, patients were included if they underwent surgical closure of a ventricular septal defect or an atrial septal defect in childhood between 1988 and 2002. Every patient completed an intelligence assessment upon inclusion, 14-27 years after surgery, using the Wechsler Adult Intelligence Scale Version IV., Results: A total of 58 patients met the eligibility criteria and were included in the analyses. No statistically significant correlation was found between blood pressure during cardiopulmonary bypass and intelligence quotient scores in adulthood (r = 0.138; 95% CI-0.133-0.389). Although amongst patients with mean arterial pressure < 40 mmHg during cardiopulmonary bypass, intelligence quotient scores were significantly lower (91.4; 95% CI 86.9-95.9) compared to those with mean arterial pressure > 40 mmHg (99.8; 95% CI 94.7-104.9)., Conclusions: Mean arterial pressure during surgery of ventricular septal defects or atrial septal defects in childhood does not correlate linearly with intelligence quotient scores in adulthood. Although there may exist a specific cut-off value at which low blood pressure becomes harmful. Larger studies are warranted in order to confirm this, as it holds the potential of partly relieving CHD patients of their cognitive deficits.

Background: Surgical repair of partial anomalous pulmonary venous connection (PAPVC) may disturb the electrical conduction in the atria. This study documents long-term outcomes, including the late occurrence of atrial tachyarrhythmia and bradyarrhythmia., Methods: This retrospective study covers all PAPVC operations at Aarhus University Hospital between 1970 and 2010. Outcome measures were arrhythmias, sinus node disease, pacemaker implantation, pathway stenosis (pulmonary vein(s), intra-atrial pathway, and/or superior vena cava), and mortality. Data were collected from databases, surgical protocols, and hospital records until May 2018., Results: A total of 83 patients were included with a postoperative follow-up period up to 46 years. Average age at follow-up was 43 ± 21 years. During follow-up, new-onset atrial fibrillation or atrial flutter appeared in four patients (5%). Sinus node disease was present in nine patients (11%). A permanent pacemaker was implanted in seven patients (8%) at an average of 12.7 years after surgery. Pulmonary venous and/or superior vena cava obstruction was seen in five patients (6%). Stenosis was most prevalent in the two-patch technique, and arrhythmia was most prevalent in the single-patch technique. Sixty-seven (81%) of 83 patients had neither bradyarrhythmias nor tachyarrhythmias or pacemaker need., Conclusions: This study contributes important long-term data concerning the course of patients who have undergone repair of PAPVC. It confirms that PAPVC can be operated with low postoperative morbidity. However, late-onset stenosis, bradyarrhythmias and tachyarrhythmias, and need for pacemaker call for continued follow-up.

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.

Objective: For the first time, we wish to assess the psychiatric burden in adult patients living with small, unrepaired atrial septal defects (ASD) using register-based data, combined with self-reported measures on levels emotional distress and educational status., Design: A descriptive study using both the unique Danish registries and validated psychiatric questionnaires and scales, including: The Symptom Checklist, Whiteley-7, and Brief Illness Perception Questionnaire., Patients: Adult patients with small, unrepaired ASD, diagnosed between 1953 and 2011., Outcome Measures: Number of register-based psychiatric diagnoses. Additionally, symptoms of anxiety, depression, somatization, health anxiety, illness perception, and levels of educational attainment compared to age- and gender-matched individuals from the reference group., Results: We identified 723 Danish patients with a small, unrepaired ASD. Approximately 17% of the patients eligible for inclusion had one or more psychiatric diagnoses. The most common diagnoses were neurotic, stress-related, and somatoform disorders. A total of 140 patients (mean age 33 years) was included for examination of their mental health using psychiatric questionnaires and scales. Patients with small ASD had higher scores of depression and anxiety when compared to the reference group (0.57 ± 0.67 vs 0.39 ± 0.52; P < .001). Furthermore, patients reported more symptoms of somatization (0.51 ± 0.48 vs 0.34 ± 0.39; P < .001). Finally, a higher percentage of patients with an unrepaired ASD had no education beyond high school when compared to the reference group (33.3% vs 14.3%; P < .001) and a smaller proportion of patients had completed a short-cycle higher education (6.5% vs 16.1%; P = .002)., Conclusion: Patients with a small, unrepaired ASD in adult life have more symptoms of anxiety, depression, and somatization compared to the reference group. Furthermore, fewer patients had no education beyond high school. These results support the guidelines that adults with small ASD warrants regular lifelong follow-up in specialized clinics aware of not only adverse somatic outcome but also rates of depression and anxiety., (© 2019 Wiley Periodicals, Inc.)

Background: Adult patients with a small, unrepaired atrial septal defect (ASD) have higher mortality and increased risk of several comorbidities compared to general population. Further, reduced submaximal and peak exercise capacity was recently demonstrated in these patients. The mechanisms behind these findings remain unexplained and, therefore, biventricular contractility during exercise was assessed by evaluating the force-frequency relationship in the same group of patients., Methods: Adults patients with a small, unrepaired ASD and healthy age- and gender-matched controls were examined using echocardiography during supine bicycle exercise. Continuous tissue velocity Doppler was used to evaluate isovolumetric acceleration (IVA) and systolic velocities during an incremental workload protocol. All data were analyzed post hoc in a blinded fashion., Results: We included 30 patients previously diagnosed with a small, unrepaired ASD (mean age 35 years, 63% female) and 25 controls (mean age 34 years, 64% female). Patients had similar values of IVA and systolic velocities at rest when compared with the healthy controls. Further, no differences in IVA was found at peak heart rate for neither the left ventricle (90 ± 39 vs 129 ± 68 cm/s 2 , P = 0.1547) nor the right ventricle (128 ± 56 vs 154 ± 56 cm/s 2 , 0.5691). There were no correlations between peak velocities and the lower exercise capacity previously reported in these patients., Conclusion: Adult patients with a small, unrepaired ASD have normal biventricular contractility at rest and during exercise when compared with healthy peers. Consequently, the pathophysiological mechanisms behind the impaired exercise capacity previously demonstrated in these patients remains unknown and will be a target for future work., (© 2019 Wiley Periodicals, Inc.)

Background: Adult patients with small, unrepaired atrial septal defects (ASD) have higher late mortality than the background population. In this nationwide study, we characterize the late natural history of adults with small, unrepaired ASD., Methods: Using the Danish National Patient Registry, we included all Danish patients, diagnosed between 1953 and 2011 with an unrepaired ASD. Additionally, all patients, aged 18-65, were invited for clinical testing. Patients also completed a general health survey for comparison with the general population., Results: We identified 723 patients with a small unrepaired ASD. Since the time of diagnosis, 182 patients had died, with an average lifespan of 63 years. The most common cause of death was heart failure. Furthermore, ASD patients had a higher burden of chronic disease than the general population (38.2% vs. 26.9%; p = 0.005), particularly lung disease (3.6% vs. 0.9%; p = 0.008). A total of 153 patients (mean age 32 y) underwent additional testing. On echocardiography an open defect was verified in 19.6% (n = 30) of the patients, of which half subsequently underwent intervention. Interestingly, 6-minute walking distance was markedly reduced (p < 0.0001 compared to normative values) no matter whether the defect was open or closed by echocardiography. Finally, 25.5% of the patients often felt stressed or nervous as compared with 16.3% of the general population (p = 0.004)., Conclusions: Patients with small, unrepaired ASD in adult life have reduced lifespan, more chronic diseases, impaired submaximal exercise capacity, and higher levels of stress than the general population. The current guidelines for intervention and follow-up may need to be reconsidered., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: Adult patients with small, unrepaired atrial septal defects have an increased risk of pneumonia, atrial fibrillation, and stroke. Furthermore, they have higher late mortality than the background population. The functional capacity is unknown in these patients. Therefore, our objective was to determine exercise capacity in adult patients diagnosed with an unrepaired atrial septal defect compared to healthy controls., Design: A cross-sectional study., Patients: Adult patients with small, unrepaired atrial septal defects, aged 18-65, diagnosed between 1953 and 2011., Interventions: Cardiopulmonary exercise test was performed using an incremental bicycle test and gas exchange was measured using breath-by-breath technique., Outcome Measures: Primary outcome was peak oxygen uptake, secondary outcome was maximal workload and ventilatory anaerobic threshold., Results: We included 32 patients previously diagnosed with a small, unrepaired atrial septal defect and 16 healthy, age- and gender-matched controls (age 36.3 ± 13 years). Patients were divided into two groups based on whether the atrial septal defect was open (age 36.3 ± 11 years) or spontaneously closed (age 36.8 ± 14 years) since time of diagnosis. No differences in demographic characteristics or weekly exercise levels were found. Both patient groups reached lower peak oxygen uptake (open: 31.7 ± 11 mL/kg/min; spontaneously closed: 29.7 ± 6.9 mL/kg/min) compared with controls (42.6 ± 6.1 mL/kg/min; P = .0001). Workload (open: 2.6 ± 1.0 watt/kg; spontaneously closed: 2.5 ± 0.6 watt/kg) and aerobic capacity (open: 21.4 ± 8.7 mL/kg/min; spontaneously closed: 22.5 ± 6.5 mL/kg/min) was also poorer in patients compared to controls (workload: 3.5 ± 0.5 watt/kg; P = .0006, aerobic capacity: 31.3 ± 6.8 mL/kg/min; P = .0007)., Conclusion: Adult patients with a diagnosis of small, unrepaired atrial septal defect have significantly impaired exercise capacity when compared to healthy controls. The impairment was present even if, by the time of assessment, the defect had closed spontaneously. The pathophysiological mechanisms behind the impaired exercise capacity demonstrated in these patients remain unexplained and will be a target for future work., (© 2018 Wiley Periodicals, Inc.)

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

Objectives: Atrial and ventricular septal defects are commonly closed surgically with low rates of post-operative morbidity, and the long-term outcome has widely been described as benign. Nevertheless, there is an increasing understanding of late morbidity, and the possibility of postoperative chronotropic impairment is continuously questioned. Furthermore, potentially abnormal chronotropic responses may be associated with the recently demonstrated overrepresentation of late arrhythmias. We conducted this review to methodically describe the potential effects of surgical defect closure on the chronotropic response to exercise., Methods: We performed a search protocol based on the 'Preferred Reporting Items for Systematic Reviews and Meta- Analyses' (PRISMA) guidelines. A data collection form was specifically developed and data were extracted from the included studies by a primary reviewer and crosschecked by a secondary reviewer., Results: A search of electronic databases revealed 16 publications, which consistently reported an abnormal heart rate responses to exercise after surgical closure. In contrast, these and other studies convincingly showed normal chronotropic response in un-operated patients as well as after percutaneous closure. Potential mechanisms include disturbances in the sinoatrial or atrioventricular conduction system and denervation in the proximity of the aorta during the surgical procedure., Discussion: ASD and VSD patients have abnormal heart rate responses to exercise after surgical closure, which indicates a need of change in the preoperative information given to these patients and their parents before surgical defect closure. Although there may not be any alternatives to surgery, patients should still be informed about potential long-term consequences, including the risk of chronotropic incompetence. Moreover, this review suggests that 'specialized', long-term follow up may be indicated.

Objectives: Atrial and ventricular septal defects are the most common congenital heart defects and the closing procedures share important similarities. Generally, the postoperative outcome is considered benign, but there is growing concern regarding late ventilatory function. Therefore, the aim of this review was to describe the ventilatory function in patients with open as well as percutaneously or surgically closed atrial and ventricular septal defects., Methods: We performed a search protocol based on the "Preferred Reporting Items for Systematic Reviews and Meta-Analyses" (PRISMA) guidelines. A data collection form was specifically developed and data were extracted from the included studies by a primary reviewer and cross-checked by the secondary reviewer., Results: We found an increasing evidence of late impairment in ventilatory efficiency, and despite that ventilatory function is commonly normal at rest, abnormalities were disclosed during exercise. There are indices that surgical closure plays an important role., Discussion: Atrial septal defect and ventricular septal defect patients have persisting, long-term impairment in ventilatory efficiency during exercise. Although the pathogenesis behind this finding may be multifactorial, there are indices that the surgical procedure may play an important role. Nevertheless, the literature is this field is sparse, and additional studies are needed.

Atrial septal defect (ASD) is associated with pulmonary hyperperfusion and inflammation and has been linked with increased risk of pneumonia. We investigated the risk of pneumonia in patients with ASD and the impact of ASD closure in a nationwide cohort study. All adults in Denmark (n = 1,168) diagnosed with ASD from 1977 to 2009 were identified through Danish public registries. We compared the risk of hospitalization for pneumonia, use of antibiotics, and mortality with age- and gender-matched general population controls. The median follow-up from time of diagnosis was 9.6 years (range 1 to 33). Cox regression analysis showed that both patients with closed (n = 863) and unclosed (n = 305) ASD had a higher risk of hospitalization for pneumonia (adjusted hazard ratios [HRs] 2.8, 95% confidence interval [CI] 2.3 to 3.5 and 3.1, 95% CI 2.4 to 4.0, respectively) than controls. Patients with ASD also used more antibiotics (adjusted HR for closure 1.4, 95% CI 1.3 to 1.5 and adjusted HR for no closure 1.4, 95% CI 1.2 to 1.6) than the controls. Antibiotic use normalized (adjusted HR 1.1, 95% CI 1.0 to 1.3) and the risk of pneumonia decreased (adjusted HR 1.8, 95% CI 1.1 to 3.0) within 5 years after closure. The proportion of patients with ASD with pneumonia-related death (8.4%) was comparable with that of the general population (9.5%). In conclusion, patients with ASD had a substantially higher risk of hospitalization for pneumonia and used more antibiotics than the general population. The pneumonia risk diminished, but did not completely normalize, 5 years after closure., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objectives: In this study, we report the first experiences with the GORE(®) Septal Occluder in transcatheter treatment of patients with atrial septal defects (ASD)., Methods: Twenty-two patients with secundum ASD were selected for GORE(®) Septal Occluder device closure. Mean age was 25.8 ± 4.6 years (range 4-60), 10 were children below the age of 15. Closure was guided by transeosophageal or intracardiac echocardiography and transthoracic echocardiography was used at follow-up (median time 3 months). All defects were balloon sized and device size was chosen according to an occluder/defect size ratio ≥2., Results: The mean ASD size was 11.4 ± 0.5 mm (range 6-15). All GORE(®) Septal Occluder sizes (15, 20, 25 and 30 mm) were used. Six patients had additional defects. The aortic rim was deficient in twelve patients and four patients had a septal aneurysm. Symptoms were seen in 17 patients and 19 had dilated right ventricles. Implantation was successful in all patients with no complications. Delivery was easy and intuitive by the new delivery system. None of the patients had residual shunts at follow-up. Mean procedure time was 38 ± 5 min (range 20-120). All patients had resolution or improvement in symptoms. There were no new onset arrhythmias or AV valve regurgitation and right ventricular dimensions were normalized., Conclusions: The GORE(®) Septal Occluder is an efficient device for closing ASDs up to 15 mm. It is feasible for defects with different anatomy including a deficient aortic rim, septal aneurysm and multiple fenestrations and due to its compliance and conformability it has a presumed low risk of erosion., (Copyright © 2013 Wiley Periodicals, Inc.)