bluuebunny/crossref_metadata_2025_split huggingface dataset:
The ventricular septal defect in tetralogy of Fallot: A multi-centre morphological and statistical shape analysis study
Authors: Sabarigirivasan V , Sivera R S , Crucean A C , Cook A C 
Published: October 2024

Abstract

Background
Ventricular septal defects (VSDs) in Tetralogy of Fallot (TOF) are often thought to be ‘large and unrestricted’ but experience suggests they have a more heterogenous morphology. Understanding the morphological variations and dimensions is the first step in modifying surgical closure or optimising potential device design.

Purpose
The objective of this study was to perform a comprehensive multi-center morphological analysis of the characteristics of the VSD in TOF. Additionally, we sought to perform a preliminary 2D shape analysis and principal component analysis (PCA) on standardised photos of the VSDs. Through this multi-dimensional approach, we aim to elucidate the morphological variations and structural characteristics of the VSD in TOF as a basis for future 3D shape analysis.

Methods
Of 495 specimens analysed, 460 were included in the morphological analysis. Standardised protocol was applied to assess the characteristics of the VSD: basic measurements of the height, width, inferior limb, and length of the inferior fibrous rim (if perimembranous) were taken and indexed to ventricular length. The degree of aortic override and presence of aorto-mitral continuity were also documented with double outlet right ventricle (DORV) being defined as more than 50% of aorta supported by the right ventricle. Statistical analysis was performed to assess variations in TOF morphology across different age groups and disease phenotypes. Standardised photographs of 130 VSDs were taken from the right ventricle and traced for PCA, in order to assess shape variations among the VSDs.

Results
Both the length of the inferior limb of the PM VSD and the length of the fibrous rim increased with ventricular length but the rate of change was lower for the fibrous rim. A chi-square analysis comparing the presence of double outlet right ventricle and the presence of a fibrous continuity between the aortic and mitral valves showed no association between these features (p = 0.89). The results of PCA in the indexed datasets showed that the largest variation was in the size of the VSD and then from the shape of the VSD where two distinct morphologies emerged: a classic V-shaped VSD and wide but shallow variant.

Discussion
Our study challenges conventional definitions and assumptions regarding the morphological characteristics of the VSD in TOF and suggests that components may change size at differing rates. Our study conclusively shows that DORV and TOF can co-exist and the definition of DORV should be made independently of the presence of aorto-mitral continuity. Contrary to the traditional view of TOF featuring large unrestricted VSDs, our findings suggest wide shallow VSDs are common. These findings challenge paradigms and underscore a necessity for the continuing morphological re-evaluation of TOF utilising large datasets with a diverse phenotypic spread.
Risk factors of 30-day mortality following cardiac surgery in adults with congenital heart disease: analysis from the Japanese registry
Authors: Osawa T , Machino T , Saji M , Sumita Y , Kanaoka K , Kawamatsu N , Ishizu T 
Published: October 2024

Abstract

Background
With advancements in pediatric therapies, the adult congenital heart disease (ACHD) population has grown consistently. ACHD patients often face complications necessitating cardiac surgery, and understanding surgical risk factors is essential.

Purpose
This study aims to assess ACHD surgical risk factors using big data, addressing evidence gaps in previous research limited to univariate analysis due to few perioperative adverse events.

Methods
We analyzed the Japanese Registry of All Cardiac and Vascular Diseases-Diagnosis Procedure Combination (JROAD-DPC) database from April 2013 to March 2020. The JROAD-DPC database covers nearly all teaching hospitals with cardiovascular beds participation in JROAD. Our study included ACHD patients (&gt;15 years) undergoing open-heart surgery. Our primary outcome was 30-day mortality. We assessed clinical backgrounds, admission status, treatment, and length of hospital stay.

Results
Out of 13,677 ACHD patients (mean age: 53 years, females 47%) undergoing cardiac surgery assessed for 30-day mortality, 13,516 (98.8%) patients were categorized into the survival group and 161 (1.2%) into the death group. Mortality rates varied by complexity of congenital heart disease (CHD): 0.8% for simple, 1.8% for moderate, and 0.9% for great. The figure shows that a significant increase in 30-day mortality rates with age was demonstrated in simple and moderate complexity (p&lt;0.001 for trend analysis in both categories). In contrast, for cases of great complexity, the increase in 30-day mortality rates with aging was not statistically significant (p=0.443). The death group was significantly older and had higher rates of ambulance admissions, renal disease, and peripheral vascular disease. Intra-aortic balloon pumps (IABP), veno-arterial extracorporeal membrane oxygenation (VA-ECMO), and dialysis during hospitalization were significantly higher among deceased patients. Length of ICU stay was significantly longer in the death group (median [IQR]; 3 [2-4] vs. 6 [3-11], p&lt;0.001). Cox proportional-hazards model showed that age, moderate complexity, emergency admission, hospital bed counts, renal disease, and use of IABP and VA-ECMO were independently associated with 30-day mortality (Table).

Conclusions
The present study, which utilizes the nationwide administrative DPC database, demonstrates that surgical outcomes for patients with ACHD, as measured by 30-day mortality rates, are generally favorable. These outcomes significantly depend on factors such as age, complexity of CHD, emergency admissions, hospital capacity, renal disease, and use of mechanical circulatory support.
Transcranial doppler to detect high-risk PFO and predict decompression illness in divers
Authors: Lee H , Lim D S , Kang Y C , Lee J Y , Oh M Y , Lee D G , Park J S , Kim C H , Jung J H , Choi R K 
Published: October 2024

Abstract

Background
It has not yet been clearly demonstrated whether it is feasible to diagnose patent foramen ovale (PFO) using transcranial doppler (TCD) and to use it in strategies to prevent decompression illness (DCI) in self-contained breathing apparatus (SCUBA) divers.

Purpose
DIVER-PFO study confirmed that high-risk PFO is associated with an increased risk of DCI in SCUBA divers. In this prespecified post-hoc analysis, we aimed to prospectively evaluate the accuracy of TCD to diagnose high-risk PFO and to predict the occurrence of DCI.

Methods
Eighty-two consecutive divers (mean age; 41 years, male 80.5%) underwent transesophageal echocardiogram (TEE) and TCD with a saline bubble test to determine the presence of PFO and were subsequently divided into high-risk and low-risk groups by TEE findings. Divers in the high-risk PFO group included those with the following conditions: PFO with atrial septal aneurysm, hypermobility, PFO size ≥2 mm, or right to left shunt at resting state. The enrolled divers were then followed-up using a self-reported questionnaire through e-mail or telephone interview for 3 years, while they were blinded to their PFO status. The primary endpoint of the study was PFO-related DCI, comprising cases of cutaneous or neurologic DCI, DCI symptoms requiring hyperbaric chamber therapy, DCI symptoms within 30 minutes after surfacing, or unexplained death occurring within 2 weeks after diving.

Results
PFO was detected in 47 (57.3%) and 59 (72.0%) divers by TCD and TEE, respectively. Diagnostic accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of TCD were 68.3%, 67.8%, 69.6% 85.1%, and 45.7% for the diagnosis of PFO, and 65.9%, 82.8%, 56.6%, 51.1% and 85.7% for the diagnosis of high-risk PFO, respectively. PFO-related DCI occurred in 9 divers in the PFO group (PFO vs non-PFO: 6.4 vs 0.7 incidences per 10,000 person dives; p=0.03) during a mean follow-up of 25.6 months. Integrated time-dependent Area Under the Curves for TCD and TEE to predict DCI occurrence using Cox’s proportional hazards regression model were 0.714 and 0.675, respectively.

Conclusions
TCD was moderately accurate to diagnose high-risk PFO in divers. However, it predicted DCI as much as TEE.Study flowIncidence of DCI in the PFO vs No PFO
Machine learning prediction for prognosis and long-term effectiveness for transcatheter ventricular septal defect closure: a 5-year single center experience
Authors: Li Z F , Li W C , Kong P X , Feng S Y , Li Y H , Li J , Liu Z Y , Zhang F W , Wang S Z , Fang F , Ouyang W B , Hu X P , Xie Y Q , Pan X B 
Published: October 2024

Abstract

Background
Transcatheter therapy, with minimal invasiveness and rapid recovery advantages, has emerged as the primary choice for treating perimembranous ventricular septal defect. However, patients receiving transcatheter occlusion may confront conduction abnormalities due to the occluder's proximity to the cardiac conduction bundle during implantation. Few have assessed the risks of postoperative conduction block (CB) via robust machine learning models.

Purpose
The purpose of this study was to evaluate our long-term follow up results of pmVSD occlusion and establish a prediction model to identify risk predictors of conduction abnormalities via robust machine learning methods.

Methods
Five methods including logistic regression with a stepwise selection of variables, lasso regularization; random forest; gradient descent boosting (GBM); and support vector machine, were used to train models for predicting risks of late-onset and persistent conduction block through 5 years of follow-up and were validated using 5-fold cross-validation. Receiver-operating characteristic curves and Brier scores were utilized to evaluate model discrimination and calibration, respectively. The top prediction variables were identified by using the best performing models, using the relative influence score of each variable in 5-fold cross-validation.

Results
The GBM model performed the best with an AUC of 0.82 (95% confidence interval [CI]: 0.75 to 0.89) for predicting late-onset CB (Brier score: 0.0204), and 0.70 (95% CI: 0.62 to 0.78) for persistent CB (Brier score: 0.0003) over entire study period. Conduction block on admission, advanced age and aortic regurgitation before discharge were strongly associated with late-onset CB, whereas decreased left ventricular ejection fraction, enlarged left ventricle end diastolic diameter at 1-month follow-up were the most significant predictors of persistent CB.

Conclusions
These models predict the risks of temporary and persistent postoperative conduction block in patients with pmVSD occlusion and shed light on preventing long-term complications.Study FlowchartCentral Illustration
Sex differences in outcomes among patients undergoing transcatheter atrial septal defect closure: Do they benefit equally?
Authors: Schrutka L , Abrahamyan L , Vishwanath V , Flores-Umanzor E , Asghar A , Benson L , Osten M , Horlick E 
Published: October 2024

Abstract

Background
Although sex differences in congenital heart disease are prevalent and relevant, there is limited research on their role in atrial septal defect (ASD) closure. We aimed to investigate sex differences in baseline characteristics, procedural and long-term outcomes of patients with transcatheter ASD closure.

Methods
Data from adult patients undergoing ASD closure between 2005-2016 were retrospectively analyzed.

Results
Of the 853 patients included, 281 (32.9%) were male and 572 (67.1%) were female. Females were more symptomatic at presentation than males (p &lt; 0.001). Males had a higher frequency of cardiovascular comorbidities than females (p &lt; 0.001). Although echocardiographic right ventricular (RV) diameter relative to body surface area was equal in males and females (2.49 cm/m2 versus 2.57 cm/m2; p = 0.072), males had more RV dysfunction (12.1% versus 7.6%; p = 0.028). In contrast, females had higher RV systolic pressures (39.71 mmHg versus 37.23 mmHg; p = 0.025), and were more likely to have moderate to severe tricuspid regurgitation (15.4% versus 8.9%; p = 0.007). Males had larger defects with use of larger implants (p &lt; 0.05). Procedure-related complications were rare and did not differ by sex. At 12-month follow-up, both male and females showed comparable decreases in RV diameter (-0.57 cm versus -0.59 cm; p = 0.751), RV systolic pressure (-4.57 mmHg versus -6.31 mmHg; p = 0.094), and severity of tricuspid regurgitation (p = 0.173; Figure). After a mean follow-up of 3 years (SD = 5), no significant differences were observed in the incidence of death (incidence rate ratio (IRR) = 0.49 [95% CI 0.27-0.83]; p = 0.402), new onset atrial fibrillation (IRR = 3.22 [95% CI 2.57-3.99]; 0.063), cardioversion or ablation (IRR = 0.40 [95% CI 0.20-0.71], p = 0.335), stroke/TIA (IRR = 0.29 [95% CI 0.12-0.56]; p = 0.745), and pacemaker implantation (IRR = 0.29 [95% CI 0.12-0.57], p = 0.728).

Conclusions
Although patient profiles differed by sex, procedural and long-term outcomes were comparable, suggesting that transcatheter ASD closure can be safely performed in both males and females.Figure.Sankey Plot
Different microRNA expression in the right ventricular outflow tract myocardium of tetralogy of Fallot
Authors: Kotani Y , Suzuki H , Yamazaki S , Shishido T , Shimizu S , Kasahara S 
Published: October 2024

Abstract

Background
When repairing tetralogy of Fallot (TOF), there are two surgical options according to the size of the pulmonary valve: transannular patch repair and valve-preserving repair. Since the development of the pulmonary valve depends on the degree of obstruction of the right ventricular outflow tract (RVOT), molecular profiles of the RVOT muscles may be associated with the surgical decision. As a recent study shows altered microRNA expression of the right ventricular myocardium even in TOF patients, microRNA expression of the RVOT infundibular muscle may be different between the two procedures.

Purpose
To evaluate differences in microRNA expression levels of RVOT infundibular muscles between the transannular patch repair and valve-preserving repair groups.

Methods
Surgically resected infundibular muscles from TOF patients are frozen in liquid nitrogen and stored in our hospital biobank. Nine samples collected between August 2020 and July 2022 were used in this study. Five patients underwent a valve-preserving repair and the others underwent a transannular patch repair. Six patients were female and mean age at operation was 453 ± 240 days. These samples were homogenized and total RNA including microRNAs were extracted. MicroRNA expression levels were measured using a microRNA array capable of detecting over 2000 microRNAs simultaneously.

Results
1171 microRNAs were detected in all nine samples. The expression levels of 22 microRNAs were significantly different between the transannular patch repair group and the valve-preserving repair group (P&lt;0.05). The expression levels of 19 microRNAs were significantly higher in the transannular patch repair group and the others were significantly higher in the valve-preserving repair group. More than 2-fold changes were observed in 2 microRNAs between the transannular patch repair and valve-preserving repair groups.

Conclusion
MicroRNA expression of RVOT infundibular muscle differs between the transannular patch repair and valve-preserving repair groups. Therefore, microRNA analysis will be useful to evaluate the pathophysiology of TOF. In addition, microRNAs may become a useful biomarker for surgical decision making in TOF.
Long-term heart failure risk after atrial septal defect closure in elderly
Authors: Ho C B , Ng P K , Lun W C , Kwok W Y , Leung C Y , Ko H Y , Kwan C M , Ho M H , Chim M Y , Yeung Y K , Leung C , Chui S F , Chan K C , Wong C Y , Lee K Y 
Published: October 2024

Abstract

Background
There is concern that atrial septal defect (ASD) closure will unmask underlying diastolic dysfunction or left heart disease in elderly causing heart failure. It is controversial whether fenestrated devices should be used in elderly patients.

Purpose
To assess the long-term heart failure risk after ASD closure in elderly and risk factors associated with heart failure hospitalization in a single centre.

Method
From 2003-2023, all elderly patients aged more that 60 years old who had ASD closure performed successfully in this centre were retrospectively studied. Baseline demographics, underlying risk factors and survival without heart failure hospitalization were analyzed.

Result
From 2003-2023, 73 patients aged more than 60 years old underwent successful ASD closure in our centre. None of these patients had balloon occlusion test for assessment of left atrial pressure. Median age was 66 years old (60-84) at operation and 27 (37.0%) of them were male. Common risk factors included diabetes (n=9, 12.3%), hypertension (n=31, 42.5%) and ischaemic heart disease (n=8, 11%). Thirty-nine (53.4%) patients had pre-existing atrial fibrillation and 17 (23.3%) of them had moderate to severe left sided valvular lesions. Heart failure symptoms were present in 31 (42.5%) of them (Figure 1). During a median follow-up time of 4 years (1-15 years), 80.8% of patients did not experience heart failure hospitalization (Figure 2). Pre-existing atrial fibrillation and left-sided valvular lesions were associated with higher risk of heart failure hospitalization (OR 4.06, 95% CI 1.03-16.06 p=0.036 and OR 4.9, 95% CI 1.41-17.09 p=0.009).

Conclusion
In a median follow-up time of 4 years, patients aged more than 60 years old had low rate of heart failure hospitalization after ASD closure. Pre-existing atrial fibrillation and left-sided moderate to severe valvular lesions were associated with higher risk of heart failure admission. Further studies should be conducted on ASD closure in these group of patients.Figure 1Figure 2
E-selectin and asymmetric dimethylarginine plasma levels in adult cyanotic congenital heart disease patients: relation to biochemical parameters, vascular function, and clinical status.
Authors: Nartowicz S , Miechowicz I , Szczepaniak-Chichiel L , Bartczak-Rutkowska A , Gabriel M , Lesiak M , Trojnarska O 
Published: October 2024

Abstract

Background and Aim
Patients with cyanosis secondary to congenital heart disease (CHD) are characterized by erythrocytosis and increased blood viscosity, which contributes to endothelial dysfunction, increased arterial stiffness, and impaired vascular function, which may affect the final clinical presentation. Asymmetric dimethylarginine (ADMA) and e-selectin (e-sel) are valuable biomarkers for prognosis of endocardial dysfunction and vascular damage. Their concentration levels in blood serum have the potential to be a rapid and easily accessible tool that reflects the severity of the disease. We aimed to assess e-sel and ADMA levels, their relationship with clinical status, as well as endothelial and vascular function.

Methods
A cross-sectional study including 36 adult CHD cyanotic patients [(17 males)(42.3 +/- 16.3 years)] with arterial blood oxygen saturation less than 92% and 20 healthy controls [(10 males)( 38.2 +/- 8.5 years)] was performed. All patients underwent clinical examination, blood testing, and cardiopulmonary tests. Endothelial function was assessed using intima-media thickness and flow-mediated dilatation (FMD). Vascular function using applanation tonometry methods was completed while considering aortic systolic pressure, aortic pulse pressure (AoPP), augmentation pressure (AP), augmentation index (AI), pulse pressure amplification (PPampl), and pulse wave velocity (PWV).

Results
Concentrations of e-sel and ADMA were significantly higher in patients with CHD than in controls (56.3 ± 13.6 ng/mL vs. 15.5 +/- 8.6 ng/mL, p&lt;0.001) and (1.48 ± 0.48 ng/mL vs. 0.46 ± 0.1 ng/mL, p&lt;0.001) respectively. E-sel levels correlated with gender, blood oxygen saturation, red blood cell, hemoglobin concentration, hematocrit, augmentation pressure, forced expiratory one second volume, forced vital capacity, and oxygen uptake. ADMA levels were found to correlate with age only.

Conclusions
E-sel level, unlike ADMA concentration, reflects the degree of severity of erythrocytosis and hypoxia and, thus, the physical status of patients with cyanotic CHD. E-selectin predicts increased augmentation pressure.Figure 1.A) ROC analysis for e-selectin
Variability in patient-reported outcomes among different congenital heart defects: a comparative analysis of 7,498 patients from 32 countries
Authors: Moons P , Kovacs A H , Goossens E , Luyckx K , Van Bulck L 
Published: October 2024

Abstract

Background
Patient-reported outcomes (PROs) may not always correlate with the complexity of congenital heart defects (CHDs), as significant variation has been observed between CHDs within complexity levels. Accurate comparisons across various types of CHDs necessitate large sample sizes.

Purpose
The objective of this study was to compare PROs across different CHDs in a large international sample.

Methods
‘Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease – International Study II’ (APPROACH-IS II) was an international cross-sectional investigation, in which 8,415 patients from 32 countries in 6 continents were enrolled (20 high; 7 upper-middle; 4 lower-middle; and 1 low income). For the comparison across different CHDs, conditions with at least 50 patients were included in the analysis. Hence, the sample consisted of 7,498 patients (median age=33y; 53.1% women). Patients completed questionnaires to measure perceived health status (RAND-12 Health Survey; EuroQOL-5D Visual Analog Scale); depressive symptoms (Patient Health Questionnaire-8); anxiety (General Anxiety Disorder-7) and quality of life (Linear Analog Scale). Generalized linear mixed models (GLMMs) were performed with center as random effect.

Results
Cyanotic heart defects were associated with the lowest scores for physical functioning, mental health, self-rated health, and quality of life (see Figure). Other CHDs found to be associated with poor PRO scores encompassed unrepaired secundum atrial septal defect, Ebstein anomaly, single ventricle, and truncus arteriosus. Conversely, the most favorable scores for all PROs, except for anxiety, were observed with isolated pulmonic stenosis. Other CHDs with favorable PRO scores comprised coarctation of the aorta, isolated small ventricular septal defect, congenital aortic valve disease, and repaired ventricular septal defect. Adjusted for sociodemographic and physiological factors, GLMMs revealed significant differences across defects, using the best score for each PRO as the reference (see Figure).

Conclusion
Substantial variation in PROs was observed among different types of CHDs. Interestingly, some complex heart defects demonstrated relatively good PRO scores, comparable to some simple defects. On the other hand, unrepaired simple defects or those with residual lesions, were associated with worse PROs.