bluuebunny/crossref_metadata_2025_split huggingface dataset:
Evaluating the safety and long-term efficacy of triple vasodilator therapy for pulmonary arterial hypertension associated with congenital heart disease
Authors: Luna R , Segura De La Cal T , Salguero R , Arribas F , Escribano Subias P , Sarnago Cebada F 
Published: November 2023

Abstract

Background
Pulmonary arterial hypertension (PAH) affects 5-10% of congenital heart disease (CHD) patients, but there is limited information on the safety and efficacy of triple combination therapy for PAH-CHD. The aim of this study was to assess the long-term effects of triple therapy (TT) with prostacyclins on PAH-CHD patients.

Methods
A retrospective, longitudinal, cohort study of patients with PAH-CHD under active follow-up in our center. All patients were receiving baseline dual therapy at maximum doses. Clinical characteristics, including functional class (FC), 6-minute walk test distance (6MWTd), and NT-ProBNP were recorded before initiating TT and during annual follow-ups during 2 years.

Results
60 patients were included (median age 41, 31 women 61%). 32 had Eisenmenger syndrome, 9 had coincidental shunts, 18 had postoperative PAH, and 1 had a significant L-R shunt. TT was initiated based on the clinical situation, existence of shunt and ease of administration (epoprostenol 20%, treprostinil 41%, selexipag 32%, iloprost 7%). A significant improvement in the 6MWTd was observed one year after the initiation of TT (p&lt;0.05), which was maintained at two-year follow-up, with an average improvement of 44 metres. An improvement of FC was observed in 79% of patients at one year, maintained at 2 years in 76%. A NT- proBNP levels decreased from the start, and reached significancy two years after the initiation of TT, with an average decrease of 199 ng/l (Figure 1). Twenty patients (33.3%) experienced side effects after drug initiation. Most were mild and well-controlled with symptomatic treatment. Only 3 (5%) had to change the administration route (Figure 2). By subgroups, patients without Eisenmenger and with pre-tricuspid defects had a more marked benefit.

Conclusions
TT has been shown to be safe and effective in patients with PAH-CHD, improving FC, 6MWTd, and NT-proBNP levels, particularly in pre-tricuspid defects and non-Eisenmenger PAH-CHD.Changes after initiation of TTType of treatment and side-effects
Long-term follow-up of transcatheter closure of congenital coronary cameral fistulas in infants and children: experience from a single center
Authors: Li Y F , Chen Z W , Wu J L , Xie Y M , Wang S S , Zhang Z W 
Published: November 2023

Abstract

Background
Transcatheter closure(TCC) has been considered as an effective treatment for anatomically suitable coronary cameral fistulas(CCF).However, limited data are available regarding long-term outcomes TCC of CCF in the pediatric population.

Purpose
The long-term efficacy and safety of TCC for the management of CCF was investigated in pediatric patients.

Methods
All pediatric patients(＜18 years old) diagnosed with CCF who were scheduled to undergo TCC between 2005 and 2019 in our center were retrospectively enrolled.CCF anatomic characteristics, procedural techniques, and follow-up outcomes were reviewed.

Results
81 patients(42 males, median age 3.24 years) with CCF underwent diagnostic coronary angiography, and 66 patients(median age 3.93 years,median weight 15Kg) underwent attempted TCC of CCF. Immediate successful device implantation was achieved in 62 patients, and immediate complete occlusion was achieved in 44 patients(44/62, 71.0%). The closure procedure was waived in 2 patients because of challenging anatomic structures, and a total of 6 periprocedural complications occurred in 5 patients, including acute myocardial infarction(n=3),procedural-related death(n=1), device embolization(n=1), and rupture of tricuspid chordae tendineae(n=1). The acute procedural success rate was 89.4%(59/66), and the acute complication rate was 9.1%(6/66). Follow-up data were collected for 58 (93.5%) out of 62 patients at a median of 9.3 years(range, 3.0-15.7 years). Among them, 10 adverse events occurred in 9 patients, including 5 follow-up complications(1 aortic valve perforation, 1 coronary thrombosis, 1 progressive aneurysmal dilation after reintervention, 2 new-onset tricuspid prolapse with significant regurgitation), and 5 closure failure with large residual shunts. The long-term adverse event rate was 17.2% (10/58). The angiographic features associated with both acute and long-term adverse were large CCF(P=0.005), and giant coronary artery aneurysm(CAA) (P=0.029).

Conclusions
TCC of CCF in infants and children appears to be safe and effective. Patients with large CCF and giant CAA may be at higher risk for both acute and long-term adverse events.Patient flow chartClosure strategies of TCC of CCF
Predictors of tricuspid regurgitation and right ventricular remodelling following percutaneous atrial septal defect closure
Authors: Goldar G , Burke B , Rajeswaran J , Mccloskey O , Blazevic P , Karamlou T , Komarlu R , Ghobrial J 
Published: November 2023

Abstract

Background
Atrial septal defects (ASD) are one of the most common congenital heart defects, and if left unrepaired, life expectancy is decreased due to pulmonary hypertension (pHTN), atrial arrhythmias, and heart failure. Residual right ventricular (RV) dilation or dysfunction despite ASD closure can lead to worse outcomes.

Purpose
There is insufficient data on predictors of poor RV remodelling following ASD closure in adults. In this study, we aimed to identify risk factors of persistent RV dilation or dysfunction despite percutaneous ASD closure.

Methods
Patients undergoing primary percutaneous ASD closure between 1/2010-6/2021 were followed for a year. Patients with hemodynamically insignificant or iatrogenic ASDs, pHTN and Eisenmenger syndrome were excluded. Non-linear multiphase mixed–effects cumulative logistics regression model was used to assess the trend of the individual’s degree of TR, RV dysfunction and size over time. This was further stratified by age group (≥40 and &lt;40). Partial dependence plot was used to study the association between age, body surface area (BSA), degree of RV and left ventricle (LV) dysfunction pre-procedurally with the likelihood of lack of RV remodelling. The association between ASD size and the likelihood of sustained RV dysfunction following ASD closure was also studied.

Results
A total of 185 patients met the inclusion criteria. The median age was 41 and 63% were female. The mean follow-up time of 3.6 months (range 1 day to 4.5 years). 249 echocardiograms were retrieved. Results showed a transient worsening of TR in the first 3 months post-ASD closure, which was more pronounced in older patients (≥40) (Figure 1). A larger ASD size was associated with a higher likelihood of early moderate or greater RV dysfunction (Figure 2). All patients had some degree of RV remodelling following the procedure. Older patients (≥40) were more likely to have RV dysfunction at the time of closure. Older age, RV and LV dysfunction, and high BSA were associated with poor RV remodelling or persistent RV dilation a year after ASD closure (Figure 3).

Conclusions
Patients who underwent percutaneous ASD closure with at least one of the following risk factors: age greater than 40, larger ASD, ventricular dysfunction, and high BSA require closer long-term follow-up given the reduced RV remodelling. Future studies are needed to elucidate the effect of poor RV remodelling on functional capacity, arrhythmic burden, and progression of TR and long-term outcomes.
Cardiopulmonary exercise testing in repaired tetralogy of Fallot and supraventricular arrythmias
Authors: Ribeiro Teixeira A R , Cardoso I , Bras P , Branco Mano T , Rito T , Silva S , Cruz Ferreira R , De Sousa L 
Published: November 2023

Abstract

Background
The arrhythmia burden in adult patients (Ps) with tetralogy of Fallot (TOF) is considerable, being a major late complication in repaired TOF. The incidence of atrial arrhythmias is relatively high and one of the main causes of morbidity. Our aim was to assess which cardiopulmonary exercise testing (CPET) parameters best correlate with supraventricular arrythmias (SVA) to potentially improve identification of high-risk Ps.

Methods
A single-center retrospective analysis was performed from 2009 to 2018 on adult Ps with repaired TOF who underwent maximal CPET. Ambulatory ECG monitoring was used to analyze the arrhythmic profile. Demographics, standard measures of CPET interpretation, and major cardiovascular outcomes were collected. Multivariate analysis for the prediction of SVA was performed using Cox Regression, by including all statistically significant variables in the univariate analysis and those considered clinically relevant.

Results
CPET was performed in 63 repaired TOF Ps (57% male, mean age of 34 ± 9 years), with a mean follow-up of 60 ± 33 months. 56% Ps had severe pulmonary regurgitation and 48% Ps underwent pulmonary valve replacement (PVR). Supraventricular arrhythmias occurred in 12 Ps (19%), mainly atrial fibrillation or atrial flutter (67%). There were no statistically significant differences regarding the clinical profile and baseline CPET parameters between both groups, namely: age (p=0.113), male gender proportion (p=0.581), proportion of Ps who underwent PVR (p=0.578), RV systolic dysfunction prevalence (p=0.854) and peak oxygen uptake (p=0.083). VE/VCO2 slope was an independent predictor of SVA (HR 1.41, CI 95% 1.08-1.84, p=0.012). Exercise capacity assessed by estimated metabolic equivalents (METS), as well as the peak heart rate (PHR) during exercise (HR 0.86, CI 95% 0.76-0.96, p=0.007), revealed a protective effect regarding SVA (HR 0.71, CI 95% 0.51-0.98, p=0.045). Neither peak oxygen uptake (p=0.090) nor cardiorespiratory optimal point (p=0.427) showed statistically significant correlation with SVA during follow-up. Ps with a PHR above 165 bpm had a significantly higher survival free of SVA (log-rank p=0.045).

Conclusion
In our population, VE/VCO2 slope was an independent predictor of SVA during follow-up, while both a higher PHR and a higher exercise capacity had a protective effect. CPET could be an accessible way of improving risk-stratification of TOP Ps and should therefore be included in their routine assessment.
E-wave propagation index as a marker for left ventricular thrombi post-ST elevation myocardial infarction
Authors: Fink P , Reindl M , Tiller C , Holzknecht M , Lechner I , Oberhollenzer F , Troger F , Mayr A , Bauer A , Metzler B , Reinstadler S 
Published: November 2023

Abstract

Background
The E-wave propagation index (EPI) is a novel echocardiographic measure for quantifying apical washout in the left ventricle (LV) and thus a promising marker of an elevated risk for LV thrombus. The association between EPI and LV thrombus has not been investigated in patients after ST-segment elevation myocardial infarction (STEMI).

Purpose
Aim was to determine, whether there is an association between EPI and LV thrombus development in STEMI patients treated with primary percutaneous coronary intervention (pPCI).

Methods
665 STEMI patients treated with pPCI included in the prospective MARINA-STEMI cohort study (NCT04113356) were investigated. LV thrombus presence was diagnosed via contrast enhanced CMR a median of 4 (interquartile range [IQR] 3–5) days post-STEMI. EPI and left ventricular ejection fraction (LVEF) were measured using transthoracic echocardiography (TTE) a median of 2 ([IQR] 2–3) days post-STEMI. The longitudinal length of the LV was measured in the apical 4-chamber view at end-diastole. Velocity time integral (VTI) of the E-wave was measured using pulsed wave doppler.

Results
5% (n=32) of the study population had a LV thrombus. Patients with LV thrombus had lower EPI values compared to patients without LV thrombus (0.92 vs 1.29, p&lt;0.001). EPI (OR 0.21, 95% CI: 0.06-0.73, p&lt;0.014), anterior infarct location (OR 17.18, 95 % CI: 2.25-131.05, p&lt;0.006) and LVEF (OR 0.91, 95 % CI 0.86-0.95, p&lt;0.001) were independently associated with LV thrombus. The optimal cut-off value for the prediction of LV thrombus prediction was an EPI &lt;1 (sensitivity 56.3% and specificity 80.4%). 13% (n=18) of patients with an EPI &lt;1 developed a LV thrombus compared to 3% (n=14) with an EPI≥1.

Conclusion
In STEMI patients treated with pPCI, a decreased EPI is a novel independent predictor for CMR verified LV thrombus. The optimal cut off for LV thrombus prediction is EPI &lt;1.Center Image EPI
Associations of Lifelong Exercise Characteristics with Valvular Function and Aortic Diameters in Subjects with a Bicuspid Aortic Valve
Authors: Schreurs B A , Hopman M T E , Bakker C M , Duijnhouwer A L , Van Royen N , Thompson P D , Van Kimmenade R R J , Eijsvogels T M H 
Published: November 2023

Abstract

Background
Whether exercise-induced hemodynamic stress accelerates the development of valvular dysfunction and aortic dilatation in subjects with a bicuspid aortic valve (BAV) is unclear.

Purpose
To examine the association between lifelong exercise characteristics (i.e. exercise volume, exercise intensity and type of exercise), valvular dysfunction and aortic dilatation in subjects with BAV.

Methods
Adults with BAV were classified as inactive (&lt;500 metabolic equivalent of task minutes per week [MET-min/wk]), active (500-1000 MET-min/wk) or highly active (≥1000 MET-min/wk) based on their reported lifetime exercise characteristics. Subjects’ lifelong predominant exercise intensity was classified as moderate (3-6 MET) or vigorous (≥6 MET) and predominant sport type as skill, power, mixed or endurance. Transthoracic echocardiography was used to assess the presence of aortic stenosis (AS) or regurgitation (AR) and to measure aortic diameters at the sinus of Valsalva (SV) and in the ascending aorta (AA). Aortic dilatation was defined as a Z-score ≥ 2.

Results
407 subjects (42±17 years old, 60% male) were included of which 133 were inactive, 94 active and 180 highly active. 61 subjects did not perform exercise, whereas 136 and 207 subjects predominantly participated in moderate or vigorous intensity exercise, respectively. Subjects performing sports were allocated to the skill (n=12), power (n=41), mixed (n=218), endurance (n=75) or non-dominant (n=24) exercise type group. Moderate-to-severe AS or AR was present in 96 (23.6%) and 81 (19.9%) subjects, respectively. SV and AA diameters were 34.8±6.6 and 36.5±8.1 mm, whereas 74 (18.2%) and 194 (47.7%) subjects had a Z-score ≥ 2. Exercise volume was not associated with valve dysfunction or aortic dilatation. Vigorous intensity exercise and mixed sports were associated with lower odds ratios for the risk of moderate-to-severe AS (ORadjusted: 0.43 (0.20-0.94) and ORadjusted: 0.47 (0.23-0.95), respectively). Exercise intensity and sport type were not associated with AR and aortic dilatation (Figure 1).

Conclusions
We found no deleterious associations between lifelong exercise characteristics, valvular dysfunction and aortic dilatation in subjects with BAV. In contrast, vigorous intensity and mixed sports were associated with a lower risk for moderate-to-severe AS. These observations suggest that lifelong exercise does not appear to induce deleterious cardiovascular effects in subjects with BAV.Figure 1.
Multiple coronary artery microfistulae are common and progress in adult patients late after Fontan operation
Authors: Tanaka T , Miki Y , Kido S 
Published: November 2023

Abstract

Background
Coronary angiograms of adult Fontan patients sometimes show the abnormal multiple coronary artery microfistulae (mCAFs) that drain directly into the ventricular chamber. They look like coronary artery fistulae or Thebesian vein. Angiographically detectable mCAFs are a rare finding sometimes associated with myocardial ischemia due to coronary steal and volume overload of ventricles in non-CHD adults. However, there are limited data concerning these mCAFs in the setting of CHD patients.

Purpose
The aim of this study is to demonstrate presence, character, and changes with growth of mCAFs in adult patients late after Fontan operation.

Methods
This study is single-center retrospective review of adults (16 years old or older) with Fontan circulation patients who had undergone cardiac catheterization for routine Fontan surveillance between 2009-2022. We assessed the presence of angiographically detectable mCAFs from ascending aortography. After identification of the characteristic cases, we examined the ventricular morphology, the portion of drainage, and compared with previous findings of angiograms. SaO2, EF of main ventricle, CVP, EDP of main ventricle, BNP, and use of pulmonary vasodilators were compared between mCAFs(+) group and mCAFs(-) group. We also reviewed clinical manifestation and findings of exercise stress ECG of the patients. Pulmonary atresia with intact ventricular septum was excluded because abnormal coronary arterial communications have been well documented in this condition.

Results
Among 90 Fontan patients who had undergone catheterization, significant mCAFs were observed in a total of 18 cases (20%). There were 10 right-dominant single ventricle, 4 left-dominant single ventricle, and 4 unbalanced two ventricles. In all cases, including left-dominant single ventricle, all fistulae drained into right ventricle cavity, never into left ventricle. The patients who underwent serial catheterizations demonstrated that the dilation and tortuosity of fistulae progressed over time. The comparison of two groups showed SaO2 was low, BNP and CVP was high significantly in mCAFs(+) group. There were no significant differences in other factors including age at catheterization and the use of pulmonary vasodilators. Among 10 patients who had underwent exercise stress ECG, only 1 patient showed ST segment depression. None had clinical history of chest pain, and arrhythmia, and ischemic event.

Conclusions
MCAFs are common and progress in adult patients late after Fontan operation. Although the clinical significance and etiology of these findings remains uncertain, particular attention should be given to subclinical Fontan failure caused by hhigh CVP, volume overload, and myocardial ischemia for the future of adult Fontan patients who have prominent mCAFs.
Adults knowledge and perception of their congenital heart disease: a single center cross-sectional study
Authors: Barbas De Albuquerque F , Ferreira Neves I , Mano T , Teixeira R , Sousa L , Ferreira R 
Published: November 2023

Abstract

Background
A great proportion of patients with congenital heart disease (CHD) reach adulthood and face daily challenges living with a chronic illness. Healthcare management of these patients is complex requiring a multidisciplinary approach. To date, few studies have focused on adult patients’ understanding of their CHD.

Aim
To assess adults’ knowledge of their CHD on 4 domains: (1) disease and treatment, (2) preventive measures, (3) physical activity, (4) reproductive issues.

Methods
One single center cross-sectional study. Patients were opportunistically recruited to participate in the study at their CHD medical visit, between October 2022 and December 2022. The authors translated Leuven Knowledge Questionnaire for CHD to Portuguese language. Patients that were willing to participate filled the survey before entering the physician’s room. Demographic and clinical variable were gathered during patients’ interview and from clinical records.
After run-in period, data were analyzed in SPSS ® for the descriptive analysis. Categorical values were reported as counts and percentages.

Results
From a total of 344 patients observed during recruitment period, 97 accepted to participate in the study. Demographic and clinical characteristics of the study population are described in Table 1 and 2. Mean age was 41 years, 60% were female, 34% have an education level above 12th grade and 74% are employed. The 3 most prevalent CHD were Tetralogy of Fallot (16%) and atrial septal defect type secundum (13%) and ventricular septal defect (10%).
Approximately half of the patients knew the name of their CHD and 41% could describe or locate the lesion on the pictured diagram. 38% could identify at least two symptoms of clinical deterioration.
Three-fourths of patients did not know what endocarditis is and only 14% could tell fever was the main symptom of the disease. The majority of patients did not know the risk factors for endocarditis.
73% of patients are aware that they should choose an occupation adequate to their physical status and 75% knew that they are allowed to engage in sexual intercourse if capable of doing so. Only 20% had adequate knowledge about the hereditary nature of their condition. 43% of women knew the risk of clinical deterioration during pregnancy.

Conclusion
This study showed our patients have high levels of education and employment. Still, large proportions of adults have lack of knowledge about important issues in vary domains.
These findings reinforce the need to improve patients’ education about their disease. In addition, it highlights the importance of guideline recommendations on multidisciplinary teams to achieve high quality of care in adults with CHD.
Determinants of NT-proBNP Levels in patients with a systemic right ventricle: a new cut-off level for risk stratification?
Authors: Tran F , Ruperti-Repilado F J , Haaf P , Lopez-Ayala P , Greutmann M , Schwerzmann M , Rutz T , Bouchardy J , Stambach H , Schwitter J , Wurstmann K , Freese M , Mueller C , Tobler D 
Published: November 2023

Abstract

Introduction
The role of N-terminal natriuretic peptide (NT-proBNP) in the risk prediction of patients with a systemic left ventricle is indisputable. However, its value in patients with a systemic right ventricle (sRV) is not well defined.

Method
Ninety-eight patients with NT-proBNP values at baseline from the randomized clinical trial SERVE were included. The correlation between baseline NT-proBNP values and parameters of bi-ventricular volumes and function quantified by cardiac magnetic resonance was assessed by means of adjusted linear regression models. The prognostic value of NT-proBNP and other established prognostic markers were assessed by means of adjusted cox proportional hazards model, survival analysis and c-statistic. The primary outcome was defined as the occurrence of either clinically relevant arrhythmia, heart failure or death.

Results
Median age [interquartile range, IQR] at baseline was 39 years [32,48] and 32% were female. Median NT-proBNP was 238 [137,429] ng/L. Coefficients of determination for the relationship between NT-proBNP levels and right ventricular end-systolic and end-diastolic volume index, and ejection fraction (RVEF) were +0.481, p&lt;0.001; +0.427, p&lt;0.001; and -0.561, p&lt;0.001, respectively. The sex and age adjusted hazard ratio [95% confidence interval, CI] for NT-proBNP values above the 75th percentile (429ng/L) showed an exponential increase (Figure) and there was a significant difference in survival comparing the group above our cut-off value and the group below it (Table). After adjusting for age and sex, the prognostic value of NT-proBNP was comparable to the prognostic value of RVEF and predicted VO2 max (C-statistic: 0.71, 0.72 and 0.71, respectively).

Conclusion
NT-proBNP levels correlated significantly with right ventricular parameters assessed by means of CMR. Measurement of NT-proBNP is a reliable and convenient tool for the prediction of adverse outcomes in patients with a sRV and using the cut-off value of 429ng/L could assist in the risk stratification for this population.