bluuebunny/crossref_metadata_2025_split huggingface dataset:
Prevalence of coronary artery anomalies in young sudden cardiac death: insights from a prospective state-wide registry
Authors: Paratz E , Van Heusden A , Zentner D , Morgan N , Smith K , Ball J , Thompson T , James P , Connell V , Pflaumer A , Semsarian C , Ingles J , Stub D , Parsons S , La Gerche A 
Published: October 2022

Abstract

Background
Coronary artery anomalies (CAAs) have been previously implicated as a major cause of young sudden cardiac death (SCD), particularly in exercise-related SCD with a reported prevalence of up to 33%.

Methods
A state-wide prospective out-of-hospital cardiac arrest registry identified all patients aged 1–50 years who experienced an SCD and underwent autopsy from April 2019 to April 2021. Rates of normal anatomy, normal variants and CAAs were identified and circumstances and cause of death for patients with CAAs examined.

Results
Of 1,477 patients who experienced cardiac arrest during the study period, 490 underwent autopsy and were confirmed to have experienced SCD. Of these 490 patients, five (1.0%) had a CAA identified with three having anomalies of coronary origin and two having anomalies of coronary course. In no cases was the CAA deemed responsible for the SCD. In two cases, severe coronary disease and intra-coronary thrombus with histological evidence of acute myocardial infarction were identified, in the third critical coronary disease was found, the fourth had an unrelated thoracic aortic dissection and the fifth had cardiomegaly in the setting of illicit drug use. Of 27 patients who experienced their SCD during exercise, only one had a CAA identified (the patient with thoracic aortic dissection).

Conclusion
In this prospective cohort of consecutive young patients with SCD who underwent autopsy, CAAs occurred in 1.0% of patients and did not cause any deaths. The role of CAAs in causing young SCD appears to be less significant than previously hypothesised.

Funding Acknowledgement
Type of funding sources: Public Institution(s). Main funding source(s): NHMRC, NHF
Changes in birth prevalence of hypoplastic left heart syndrome over the past half-century in Sweden
Authors: Giang Kok W , Mandalenakis Z , Fedchenko M , Eriksson P , Rosengren A , Norman M , Hanseus K , Dellborg M 
Published: October 2022

Abstract

Background
Over the past decades improvements in surgical treatment and medical management have resulted in increased survival of children with congenital heart disease, where the most complex congenital heart diseases are now also suitable for treatment. Trends in live birth prevalence in one of the most complex congenital cardiac defects, hypoplastic left heart syndrome (HLHS) have not been reported.

Purpose
The aim of this study was to assess trends in live-birth prevalence of patients with hypoplastic left heart syndrome over the past 50 years in Sweden.

Method
The Swedish National Patient and Cause of Death register were linked to estimate the annual rates of HLHS among live-born infants from 1970 to 2017. Information on number of livebirths per year in 1970–2017 was obtained from Statistics Sweden.

Results
A total of 838 infants with HLHS were identified during 1970–2017. The annual number of live born patients varied from 8 to 40 cases with a prevalence of 5.4 to 14.5 cases per 100,000 live births in the 1970s, increasing to a peak of 33.9 cases per 100,000 in 1993 and then decreasing from the late 1990s to reach an average level of 11.2 cases per 100,000 live births in the period 2014–2017.

Conclusion
The live-birth prevalence for HLHS increased to nearly six times of that in the 1970s during the 1990s. However, a subsequent decline was observed. This evolution most likely reflects a combination of increased tendency in recording the diagnosis as improved treatment becomes available and a decline related to antenatal diagnosis and termination of pregnancies.

Funding Acknowledgement
Type of funding sources: Public grant(s) – National budget only. Main funding source(s): The Swedish state under an agreement between the Swedish government and county councils (the ALF agreement, grant numbers: 236611 and 917361), the Swedish Heart-Lung Foundation (grant number: 20180644).
Long-term mortality from birth in individuals with and without isolated congenital aortic stenosis: a nationwide, register-based cohort study
Authors: Magnusson C , Mandalenakis Z , Dellborg M , Eriksson P , Fedchenko M , Rosengren A , Skoglund K 
Published: October 2022

Abstract

Background
Congenital aortic valve stenosis (CAVS) accounts for almost 5% of congenital heart defects. Increased survival has been reported in patients with complex congenital heart defects over the last decade. However, data on the long-term outcomes of simple defects such as in isolated CAVS, are still limited.

Purpose
The present study aimed to investigate the risk of mortality in patients with isolated CAVS over the last half century in Sweden.

Methods
We used data from the Swedish National Patient and Cause of Death Register to identify patients with isolated CAVS born between 1970 and 2017. Each CAVS was matched with 10 controls without congenital heart disease from the Total Population Register, according to sex and birth year. The maximal follow-up time was from birth up to 47.5 years of age. We estimated mortality rates for cases and controls, and the 95% confidence interval (CI) of their ratio. Kaplan-Meier curves were used to estimate the survival for cases and controls under the follow-up.

Results
A total of 1,258 patients with isolated CAVS and 12,550 matched controls were included in the study. The median follow-up was 24.5 years (interquartile range (IQR):18.0) for cases and 25.5 years (IQR: 17.6) for controls respectively. The overall risk of mortality was more than three times higher for patients with isolated CAVS, compared with matched controls, hazard ratio (HR) 3.1 (95%, CI 2.1–4.5). Survival at 47.5 years of age was 94.1% for cases and 97.3% for controls. Within the first year of life, the mortality risk in patients with isolated CAVS was more than 19 times higher (95%, CI 8.8–43.6) than controls. By contrast, when diagnosed with isolated CAVS above the age of 10 years, the risk of mortality was 2.1 times higher (95%, CI 1.1–3.5) compared to controls.

Conclusions
The mortality in patients with isolated CAVS was more than three times higher compared with matched controls. The highest mortality was found in patients with a diagnosis of isolated CAVS diagnosed within the first year after birth, verifying the critical form of the disease. Furthermore, patients diagnosed with isolated CAVS later in life (hence clinically assumed as a mild form of valvulopathy) have a mortality risk twice as high as controls, highlighting the need for follow-up and lifetime management, even in the mild forms of valvulopathies.

Funding Acknowledgement
Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Swedish state under an agreement between the Swedish Government and county Councils (ALF)
Risk of cardiac complications during pregnancy in patients with cardiac devices
Authors: Cantalapiedra Romero J , Pijuan-Domenech A , Galian-Gay L , Miranda-Barrios B , Serrano-Sanchez B , Goya-Canino M , Maiz-Elizaran N , Gordon-Ramirez B , Gonzalez-Fernandez V , Francisco-Pascual J , Rodriguez-Silva J , Pascual-Gonzalez G , Rivas-Gandara N , Dos-Subira L , Ferreira-Gonzalez I 
Published: October 2022

Abstract

Background
Pregnant women with cardiac devices (pacemaker or defibrillators [ICD]) are increasing in developed countries. Risk stratification in these patients is challenging, since presence of cardiac devices are not considered specifically neither in mWHO nor CARPREG II scales. In addition, the previous evidence is scarce and heterogeneous, based mostly on observational studies published years ago.

Purpose
To describe the intrinsic risk for cardiovascular complications of pregnant women with cardiac devices.

Methods
We designed a prospective study including all pregnancies under follow-up in our Heart Disease and Pregnancy Unit between January 2013 and December 2020, selecting those patients with an intracardiac device at the time of pregnancy. Following institutional review board approval, data were collected from medical records in order to classify the patients according to the type of heart disease and the mWHO scale. CARPREG II scale was calculated retrospectively from 2014 to 2018 and prospectively afterwards. All cardiological complications were identified during each pregnancy and puerperium, defined as the appearance of heart failure, arrhythmias or thrombotic complications.
Since our patients were classified in mWHO groups 2 or 2–3, we compared the incidence of complications between our cohort of patients with devices and the rest of the pregnancies of mWHO 2–3 patients without devices. We also included in the analysis patients with an unspecified mWHO. A mWHO IV patient was excluded from analysis.

Results
A total of 323 pregnancies were identified, of which 15 were in patients with devices (4.6%) and 135 in mWHO 2–3 patients without devices (41.8%). There were 9 pregnancies in 6 pacemaker carriers and 6 in 6 ICD carriers. The mean age was 34.3 years. None of them had other cardiovascular risk factors. A total of 2 cardiac complications were identified: one patient in the pacemaker group and another in the ICD group presented with a lead thrombosis at 25 and 30 weeks respectively. In both patients, anticoagulation with weight-adjusted enoxaparin was started. In the other hand, in the mWHO 2–3 patients without devices we identified 2 complications in the form of heart failure episodes: one during third trimester and another in puerperium.
When comparing our device patient group (n=14) with the mWHO 2–3 patients without devices (n=135), we observed a statistically significant higher incidence of complications in the device group (14.3% vs 1.5%, p=0.002).

Conclusions
Pregnant women with cardiac devices have a higher risk of complications than pregnant women with a similar risk profile according to the mWHO scale, mainly in the form of lead thrombosis.

Funding Acknowledgement
Type of funding sources: None.
Prevalence and subtypes of interatrial communications in 12,718 newborns
Authors: Blixenkrone Moeller E , Dannesbo S , Dehn A M , Pihl C , Sillesen A , Voegg R O B , Axelsson Raja A , Colan S , Mertens L , Vejlstrup N , Bundgaard H , Iversen K 
Published: October 2022

Abstract

Introduction
Patency of the oval foramen (PFO) and atrial septal defects (ASDs), i.e. interatrial communications, have been reported to be present in 24–92% of newborns. For isolated ASDs, the reported prevalence varies from 0.3–2.4 per 1000 livebirths. This broad range partly reflects the absence of a clear and universal clinical classification of interatrial communications. On this basis we have recently proposed a clinically applicable echocardiographic algorithm for classification of interatrial communications (figure 1), which proved superior to standard assessment by experts in terms of inter- and intraobserver agreement.

Purpose
To determine the prevalence of interatrial communications in newborns.

Methods
Echocardiograms (TTEs) from newborns (0–30 days) included in a population study (N&gt;25,000) were assessed/analyzed for interatrial communications according to the new algorithm and categorized into three PFO subtypes and three ASD subtypes.

Results
TTEs from 16,420 newborns were analyzed; 3,694 (22.5%) were excluded due to suboptimal image quality and 9 (&lt;0.1%) were excluded due to concurrent severe congenital heart disease. Of the remaining 12,718 included newborns (median age 12 days [8; 15], 48.1% female), an interatrial communication was present in 10,033 (78.9%) cases; 9,274 (72.9%) cases were classified as PFO while 759 (6.0%) were classified as ASD. In the ASD group, 368 (48.5%) had a defect size ≥4 mm, 364 (48.0%) had multiple interatrial communications, and 27 (3.6%) had the defect located in the lower 1/3 part of the septum (figure 2).

Conclusion
An interatrial communication was present in almost 4 out of 5 newborns. ASD was diagnosed in 6% of the newborns and the prevalence of PFO was 12 times higher than the prevalence of ASD. Follow-up studies of these children are expected to provide clinically useful information on the long term structural and hemodynamic impact of these well categorized ASD and PFO subtypes.

Funding Acknowledgement
Type of funding sources: Foundation. Main funding source(s): Candy's Foundation, The Danish Children's Heart Foundation
Single predictor of residual right-to-left shunt to optimally select patients for suture-mediated percutaneous patent fossa ovalis closure
Authors: Sgueglia G A , Gaspardone C , De Santis A , D'Ascoli E , Piccioni F , Iamele M , Giannico M B , Leonetti S , Gaspardone A 
Published: October 2022

Abstract

Background
In patients with patent fossa ovalis (PFO) and paradoxical embolism, percutaneous closure of the interatrial communication has been proven more effective than medical treatment only to reduce recurrent thromboembolic events. Percutaneous suture-mediated PFO closure has been proved to be a safe and advantageous alternative to device-based PFO closure, yet its overall success rate is slightly lower in unselected patients. Hence, it is extremely important to define baseline features associated with unsatisfactory results to appropriately select patients suitable for this technique.

Purpose
Systematic assessment of PFO anatomy in the largest series of consecutive patients undergoing suture-mediated percutaneous PFO closure to identify a single baseline predictor of significant residual right-to-left shunt (procedural failure) for optimal selection of patient to be submitted to this procedure.

Methods
Pre-procedural transesophageal echocardiogram (TEE) of 302 consecutive patients (113 men, 45±12 years) who underwent percutaneous suture-mediated PFO closure at a single institution were accurately reviewed to assess a series of parameters: presence and grade of spontaneous right-to-left shunt (RLS), PFO length and width, presence of atrial septal aneurysm and its maximal bulge, and presence of an embryonic or fetal remnant (Chiari network or Eustachian valve).

Results
At echocardiographic follow-up (3–6 months from the closure procedure), a residual RLS ≥2 was found in 60 (19.9%) patients. At multivariable analysis, only two anatomical variables measured at pre-procedural TEE were found as independent predictors of residual RLS ≥2 at follow-up: PFO maximum width (OR 1.89, 95% CI 1.16–3.40, p=0.019) and PFO minimum length (OR 0.58, 95% CI 0.35–0.88, p=0.018). An index based on the ratio of PFO maximum width to PFO minimum septal overlapping (W/SO) was found to be the most powerful predictor of RLS ≥2 at follow-up (OR 48.1, 95% CI 9.3–352.2, p&lt;0.001). The ROC curve for the W/SO ratio was found to have an AUC of 0.84 (95% CI 0.75–0.93) and a cut-off value of 0.61 yielding a sensitivity of 80% and specificity of 78% with a negative predictive value of 94%.

Conclusions
Baseline pre-procedural TEE assessment provides essential information for the selection of patients most suitable to undergo suture-mediated PFO closure. Our results indicate that the ratio between the maximum amplitude of the PFO and the minimum overlap of the septa is the optimal single baseline index to optimally select patient for an effective percutaneous PFO closure.

Funding Acknowledgement
Type of funding sources: None.
Cardiac remodelling in adolescent athletes – sex differences progress through adolescence
Authors: Forsaa M , Bjerring A W , Haugaa K , Smedsrud M K , Sarvari S , Landgraff H W , Hallen J , Edvardsen T 
Published: October 2022

Abstract

Background
Cardiac remodelling beyond reference values is well-known in adult athlete's heart. Male endurance athletes are more prone to develop cardiac chambers and wall thickness above reference values. Cardiac remodelling is also described in adolescent athletes, but considered to be moderate compared to adults. However, few studies evaluate adolescent athlete's heart by paediatric echocardiographic reference values. Current paediatric reference values are sex-unspecific and do not include exercise data. The impact of sex and exercise on remodelling in adolescent athlete's heart remains unclear.

Purpose
We aimed to study the development of cardiac remodelling and potential sex differences in adolescent athletes. We hypothesized that male adolescent athletes would display greater degree of remodelling compared to female adolescent athletes.

Methods
Male (M) and female (F) adolescent cross-country skiers were recruited in a longitudinal cohort study. They were examined with echocardiography at age 12, 15 and 18. Data on exercise was collected at all examinations. We evaluated echocardiographic parameters by paediatric reference values (Z-score: number of standard deviations above estimated mean in the given body surface area). Echocardiographic measures were considered above upper reference value if Z-score was ≥2.

Results
Seventy-six athletes were examined at age 12 (48 M, 28 F), 48 at age 15 (34 M, 14 F) and 34 at age 18 (23 M, 11 F). Although Z-scores were within reference values at age 12 (Table 1), a subset of athletes displayed Z-scores ≥2 for end-diastolic intraventricular septum diameter (IVSd, M 13/48=27%, F 5/28=18%) and left ventricular posterior wall thickness (LVPWd, M 6/48=13%, F 2/28=14%). The male group demonstrated enlarged left ventricular mass (LVM) from age 15 (Figure 1). Males had greater left ventricular end-diastolic volume (LV EDV) from age 12. Additional sex differences were evident from age 15 for IVSd, LVPWd and LVM (Table 1). There was no sex difference in exercise hours. Both groups had normal myocardial function through the study period.

Conclusion
Cardiac remodelling beyond reference values was observed in athletes of both sexes from early adolescent age. Sex differences were evident from age 12 with further progression. Pathological values for LVM were more frequent in males. These findings suggest that sex differences in exercise-induced cardiac remodelling is more prominent in adolescents than previously reported. Sex and exercise history should be considered in questions of pathology.

Funding Acknowledgement
Type of funding sources: Public Institution(s). Main funding source(s): The South-Eastern Norway Regional Health AuthorityCentre for Children and Youth Sport, Norwegian School of Sport Sciences
A novel method to identify an intramural segment in interarterial anomalous coronary arteries on CT-angiography
Authors: Koppel C J , Verheijen D , Kies P , Egorova A D , Lamb H J , Voskuil M , Jukema J W , Koolbergen D R , Hazekamp M G , Schalij M J , Jongbloed M R M , Vliegen H W 
Published: October 2022

Abstract

Background/Introduction
An anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an interarterial course can be assessed using Computed Tomography Angiography (CTA) for the presence of high-risk characteristics associated with sudden cardiac death. These features include a slit-like ostium, acute angle take-off, and degree of proximal luminal narrowing. However, no robust CTA criteria currently exist to determine the presence of an intramural segment.

Purpose
The aim of this study is to deduct a method to accurately identify an intramural course of interarterial ACAOS on CTA imaging.

Methods
All consecutive adult patients with an interarterial ACAOS that were evaluated at the two academic hospitals between January 2010 and July 2019 were screened for inclusion. Inclusion criteria were availability of a preoperative CTA-scan (0.5–1mm slice-thickness) and peroperative confirmation of the intramural segment. Using multiplanar reconstruction of the CTA, the distance between the lumen of the aorta and the lumen of the ACAOS (defined as “interluminal space” (ILS)) was assessed at 2mm intervals along the intramural segment (Figure 1).

Results
Twenty-five patients (64% female, mean age 46 years, 88% right ACAOS) were included. Analysis showed a mean ILS of 0.69mm±0.15mm at 2mm from the ostium. At the end of the intramural segment where the ACAOS becomes non-intramural, the mean ILS was significantly larger (1.27±0.29mm, p&lt;0.001) (Figure 2). Interobserver agreement evaluation showed good reproducibility of ILS (intraclass correlation coefficient 0.77, p&lt;0.001). ROC-analysis demonstrated that at a cut-off ILS of ≤0.95mm, an intramural segment can be diagnosed with 100% sensitivity and 84% specificity.

Conclusion(s)
The ILS is introduced as novel and robust CTA parameter to identify an intramural course of interarterial ACAOS. An ILS of ≤0.95mm is indicative of an intramural segment with 100% sensitivity and 84% specificity.

Funding Acknowledgement
Type of funding sources: None.
Strategies for thromboprophylaxis in Fontan circulation: a network meta-analysis
Authors: Van Den Eynde J , Possner M , Alahdab F , Veldtman G , Goldstein B , Rathod R , Hoskoppal A , Saraf A , Feingold B , Alsaied T 
Published: October 2022

Abstract

Introduction
The Fontan circulation is associated with an increased risk of thromboembolic events. However, the optimal strategy for thromboprophylaxis in this population remains unknown.

Purpose
The aim of this study was to compare the efficacy and safety of aspirin, warfarin, and non-vitamin K antagonist oral anticoagulants (NOACs) in a network meta-analysis using no thromboprophylaxis as a common comparator.

Methods
A comprehensive search was performed in Medline, Embase, Cochrane, and Scopus to identify relevant observational studies and randomized controlled trials published by February 14th, 2022. The primary efficacy outcome was thromboembolic events at the longest available follow-up, while major bleeding was a secondary safety outcome. Frequentist network meta-analyses were conducted to estimate the incidence rate ratios (IRRs) with corresponding 95% confidence intervals (CI) of both outcomes. Finally, ranking of treatments was performed based on probability (P) score.

Results
A total of 25 studies were included, encompassing 26,823 patient-years (aspirin: 11,948; warfarin: 11,354; NOAC: 346; no thromboprophylaxis: 3,175). When compared to no thromboprophylaxis, NOACs (IRR 0.12, 95% CI 0.03–0.41), warfarin (IRR 0.23, 95% CI 0.14–0.36), and aspirin (IRR 0.26, 95% CI 0.16–0.40) were all associated with significantly lower rates of thromboembolic events. However, the network meta-analysis revealed no significant differences in the rates of major bleeding for any of the strategies, although bleeding risk was numerically higher with NOACs and warfarin (NOAC: IRR 1.45, 95% CI 0.28–7.43; warfarin: IRR 1.38, 95% CI 0.41–4.69; and aspirin: IRR 0.72, 95% CI 0.20–2.58, Figure 1). Rankings, which simultaneously analyze competing interventions, suggested NOACs as the best strategy to prevent thromboembolic events (P score 0.919), followed by warfarin (P score 0.615), aspirin (P score 0.466), and no thromboprophylaxis (P score 0.001).

Conclusions
NOACs were associated with the greatest reduction in thromboembolic events with no significant difference in major bleeding when compared with warfarin and aspirin.

Funding Acknowledgement
Type of funding sources: None.