bluuebunny/crossref_metadata_2025_split huggingface dataset:
Decreased clinical performance in TGA-ASO patients after RVOT re-interventions; multicenter European collaboration
Authors: Engele L , Gonzalez-Fernandez V , Ruperti J , Buendia F , Rueda J , Gabriel H , Bouchardy J , Schwerzmann M , Possner M , Greutmann M , Gallego P , Ladouceur M , Tobler D , Dos L , Bouma B J 
Published: November 2023

Abstract

Background
In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT reinterventions. However, data on the impact of reinterventions on clinical performance is scarce. Therefore, we aimed to assess in TGA-ASO patients the cardiopulmonary exercise capacity (CPET) and right ventricular function stratified for the number of RVOT reinterventions.

Method
This multicenter observational study was performed in adult (≥16 years) TGA-ASO patients from the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). All patients underwent a comprehensive clinical workup including CPET and echocardiography, including right ventriculo-arterial (VA) coupling (defined as tricuspid annular plane systolic excursion (TAPSE) / right ventricular systolic pressure (RVSP) ratio). Patients were stratified for the number of reinterventions at the RVOT, including re-interventions at the pulmonary valve, main pulmonary artery and pulmonary branches. Association between RVOT re-intervention(s) and functional parameters, including predicted (%) peak oxygen uptake (peak VO2), TAPSE, right ventricle (RV) S’ and VA coupling of the RV were analyzed with linear regression.

Results
A total of 449 TGA patients were included in this study, with a mean age of 25.4 ± 5.6 years and median time until ASO was 8 days [IQR 6-15]. Eleven (2.5%) patients with previous re-interventions at the left ventricular outflow tract (LVOT) or aortic arch were excluded from the analysis. Twenty-six percent of the included patients had a ventricular septal defect (VSD) and 1.6% were diagnosed with double outlet right ventricle (DORV). In the total cohort, 13% of the patients had 1 previous RVOT re-intervention, 8% had 2 RVOT re-interventions and 5% had 3 RVOT re-interventions. Patients had echocardiographic examination and 55% underwent CPET. Predicted VO2peak was significantly reduced in patients with 1 RVOT re-intervention (72%), 2 RVOT re-interventions (69%) and 3 RVOT re-interventions (71%) compared to patients without any-reintervention (77%) (p=0.0268). Right VA coupling was a significant predictor (p=0.017) for reduced hemodynamic performance in patients with 1 RVOT re-intervention (0,50), 2 RVOT re-interventions (0,51) and 3 RVOT re-interventions (0,26).

Conclusion
In TGA-ASO patients, RVOT reinterventions were associated with a reduced clinical performance. VA coupling of the right ventricle was the most sensitive marker for hemodynamic performance in TGA-ASO patients.
Safety and efficacy of apixaban for the prevention of thromboembolism in adults with congenital heart disease and atrial arrhythmias: a prospective cohort study with historical control comparison
Authors: Kartas A , Papazoglou A , Moysidis D , Kosmidis D , Baroutidou A , Despotopoulos S , Doundoulakis I , Koutssakis A , Liori S , Apostolopoulou S , Frogoudaki A , Tzifa A , Karvounis H , Ziakas A , Giannakoulas G 
Published: November 2023

Abstract

Introduction
In adults with congenital heart disease (ACHD), atrial arrhythmias (AA) confer an increased risk of thromboembolic events. Data regarding treatment with non-vitamin K oral anticoagulants (NOACs) of patients with ACHD and AA are limited.

Methods
A prospective, multicenter, observational study of ACHD patients with AA (atrial fibrillation, atrial flutter, or intra-atrial re-entrant tachycardia) treated with apixaban from 2019 to 2022 was performed (PROTECT-AR, NCT03854149). Comparisons were made with a historical vitamin-K antagonist (VKA) treatment cohort from 2002 to 2014. The primary efficacy endpoint was the composite of stroke or thromboembolism. The primary safety endpoint was major bleeding.

Results
The apixaban-treated cohort consisted of 215 patients (median follow-up 28 months) and the historical VKA-treated ­cohort consisted of 229 patients with ACHD and AA (median follow-up 48 months). The burden of age and comorbidities was higher in the apixaban versus the VKA treatment group (Table). Comparing the apixaban with the VKA treatment group yielded no significant differences in the annualized rate of stroke or thromboembolism (1.2% vs. 1.4%, respectively; p=0.24) and major bleeding (1.2% vs. 4.4%, respectively; p=0.08) (Figure).

Conclusion
In ACHD patients with AA treated with apixaban as part of routine care, the risk of major thromboembolic and bleeding events was low. This risk was comparable with historical cohort data on VKA. Prospective studies directly comparing NOAC and VKA-treated patients are needed.
Safety of cardiopulmonary exercise testing in a large contemporary cohort of adults with congenital heart disease: implications on levels of test supervision
Authors: Breviario S , Krishnathasan K , Ministeri M , Kempny A , Frisiras A , Rafiq I , Heng E L , Barradas-Pires A , Mccabe C , Constantine A , Dimopoulos K 
Published: November 2023

Abstract

Background
Exercise intolerance is common in adults with congenital heart disease (ACHD). Cardiopulmonary exercise testing (CPET) is used routinely as an objective assessment of the exercise capacity, the severity and mechanisms of exercise intolerance, and the effect of intervention. Little is known on the safety of CPET in ACHD, resulting in the American Heart Association recommending physician supervision of all patients with intracardiac shunts.

Purpose
To assess the safety of CPET in ACHD.

Methods
We retrospectively examined 9309 CPETs performed on ACHD patients in a tertiary referral Centre over 20 years to document major or minor events. All tests were performed in-hospital, with an emergency alarm system on site, and supervised routinely by 2 clinical exercise physiologists. Major events were classified as death within 48 hours and cardiac arrest, syncope, sustained/symptomatic ventricular tachycardia (VT) and sustained/symptomatic supraventricular tachycardia (SVT) during the test. Minor events were classified as non-sustained/asymptomatic VT, non-sustained/asymptomatic SVT, bradyarrhythmia, chest pain and significant ST segment ECG changes, new onset of left or right bundle branch block).

Results
During the study period, 9309 CPETs were performed on 4941 ACHD patients (age 33.0±14.7 years, 52.3% male), of varying complexity by Bethesda classification (18.3% simple, 51.2% moderate, 27.4% great) Figure 1. A major event occurred in 56 (0.6%) tests and a minor event in 870 (9.3%) tests. There were no deaths within 48 hours, nor cardiac arrests during the test. There were 6 (0.1%) cases of sustained or symptomatic VT, 4 (&lt;0.1%) syncopal episodes and 46 (0.5%) instances of sustained or symptomatic SVT. A total of 31 (0.3%) episodes of bradyarrhythmia, 377 (4%) significant ECG changes and 267 (2.8%) episodes of chest pain were recorded. Moreover, there were 107 (1.2%) cases of non-sustained or asymptomatic VT and 88 (1%) cases of non-sustained or asymptomatic SVT. Patient fatigue (3701, 40%), shortness of breath (2246, 24.1%) and leg discomfort (1286, 13.8%) were common reasons for stopping the test, whereas only 95 (1%) tests were stopped due to chest pain.

Conclusions
Our data on a large ACHD population undergoing CPET demonstrate a potential risk for arrhythmic complications during exercise and recovery, mostly due to sustained or symptomatic SVT, while the risk of all the other major cardiovascular events is low. Our data support the use of CPET in routine ACHD practice, with quick recognition and management of potential adverse events in a controlled environment by appropriately trained cardiac physiologists.
Adult patients with univentricular physiology after Fontan palliation: prevalence and percutaneous closure of veno-venous collaterals
Authors: Nederend M , Egorova A D , Kies P , Stoger J L , Vliegen H W , Van Der Kley F , Schalij M J , Jongbloed M R M 
Published: November 2023

Abstract

Background
The Fontan operation resulted in improved survival of patients with congenital heart defects not equipped to sustain a biventricular circulation. Long-term complications are common. Veno-venous collaterals (VVC) can have both potentially beneficial and adverse physiological effects. The aim of this study was to evaluate the prevalence of VVC in adult Fontan patients and describe the percutaneous treatment strategy and the short term follow-up.

Methods
In this single-centre retrospective observational cohort study, consecutive adult Fontan patients under follow-up in the period between 2017-2023 were included.

Results
From a cohort of 51 Fontan patients, 14 (28%) underwent a percutaneous closure of VVC (71% female, mean age at intervention 24±4 years). Mean follow-up duration was 2.2±1.1 years. Indications were symptoms and/or significant exercise-related hypoxia. Ten patients (71%) had a systemic left ventricle, and 79% had an extracardiac tunnel (median conduit size 16[16-20]mm). Mean Fontan pressure was 10±4mmHg. Seven patients (50%) had Fontan-associated liver disease.
A total of 24 VVC were occluded using coils and/or vascular plugs. No periprocedural complications occurred. The VVC originated from right-sided tributaries of the vena cava inferior (36%), superior vena cava (28%) or both (36%), with the majority draining directly into the pulmonary venous circulation. Eight patients (57%) reported a reduction of symptoms. Mean saturation in rest improved significantly, yet saturation during maximal exercise remained unchanged (94±3 to 97±3%, p=0.002 and 88±3 to 91±4%, p=0.103, respectively).

Conclusions
VVC are highly prevalent in the adult Fontan population and typically connect the right-sided tributaries of the vena cava inferior and/or superior with the pulmonary veins. Low Fontan pressures do not exclude the presence of VVC. Percutaneous closure of VVC is technically feasible and safe and is associated with reduction of symptoms and a significant rise in resting peripheral oxygen saturation. These results warrant further studies.
Subcutaneous implantable cardioverter-defibrillator in adults with congenital heart disease
Authors: Njoo R , Lennerz C , Friedrich L , Bock M , Fetcu A , Schaarschmidt C , Kolb C , Tutarel O 
Published: November 2023

Abstract

Background
Subcutaneous implantable cardioverter-defibrillators (S-ICD) are an established treatment option for the prevention of sudden cardiac death (SCD). However, data in adults with congenital heart disease (ACHD), especially in direct comparison to other patient groups (e.g. cardiomyopathies) are scarce.

Methods
Retrospective single-center study of all patients receiving a S-ICD in the time-period 2012-2020. ACHD patients were compared to non-ACHD patients. Primary outcome was all-cause mortality. Secondary outcome was a combined endpoint of all-cause mortality and appropriate shocks.

Results
Altogether, 79 patients (11 ACHD, 68 non-ACHD patients) with a mean age of 40.6 ± 16.0 years (29.1% female) were included. There was no significant difference for age and sex between both groups. Secondary prevention of SCD was more often the indication for a S-ICD in ACHD (81.8%) than in non-ACHD patients (48.5%; p=0.040). Betablockers were more commonly used in ACHD patients (100% vs. 64.7%; p=0.018). There was no significant difference for other heart failure medications.
During a median follow up of 3.4 years [IQR 1.7-5.2], the primary endpoint occurred in 36.4% of ACHD vs. 2.9% of non-ACHD patients (p&lt;0.001). The secondary endpoint occurred also more often in the ACHD patient group compared to non-ACHD patients (54.5% vs. 17.6%, p=0.007). There was a numerically higher number of inappropriate shocks in the ACHD group (27.3% vs. 9.0%), albeit without a statistically significant difference (p=0.078).

Conclusions
In ACHD patients with a S-ICD, mortality as well as a combined endpoint of mortality and appropriate shocks were more common than in non-ACHD patients. The numerically higher number of inappropriate shocks deserves further attention.
Pulmonary artery aneurysm in pulmonary arterial hypertension associated with congenital heart disease
Authors: Luna R , Segura De La Cal T , Jimenez Lopez-Guarch C , Salguero R , Arribas F , Escribano Subias P , Sarnago Cebada F 
Published: November 2023

Abstract

Introduction
Pulmonary artery aneurysms are a common finding in patients with congenital heart disease, often asymptomatic and with a described low complication rate. The aim of this study is to assess the prevalence of PAA in a population with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) and associated complications.

Methods
All adult patients with PAH-CHD at our institution with angiography of the pulmonary tree by computed tomography (CT) or magnetic resonance imaging (MRI) were included in a retrospective analysis. PAA was defined as a main PA diameter &gt; 40 mm.

Results
In this study, the frequency of PAA was as high as 76%. Simple pre-tricuspid lesions had larger diameters than post-tricuspid lesions (p=0.004), but there were no significant differences with complex lesions. Also, no differences were found between the median diameters of the different PAH-CHD groups. No relationship was seen between the development of aneurysms or larger diameters and haemodynamic severity, even a majority of patients had severe pulmonary arterial hypertension.
15 patients (25% of total with PAA) had complications related to PAA: 4 patients with PA dissection (isolated or with associated thrombosis), 7 patients with chronic pulmonary thrombosis and 5 patients with left main coronary artery compression (figure 2). There were no complications in patients with diameters less than 40 mm. During a median follow-up of 3.8 years (IQR 1.6 to 6.4 years) 24 patients (30%) died or needed lung/cardiopulmonary transplantation (17 and 7 respectively). Only two (8.3%) of the patients with final events did not have PAA. Survival analysis (figure 1) showed a higher rate of death or need for transplantation in patients with pulmonary artery aneurysm, especially if there was an added complication (log-rank test, P=0.017).

Conclusions
Pulmonary artery aneurysms in patients with pulmonary arterial hypertension associated with congenital heart disease are common. The rate of related complications is also high in this population and may have prognostic implications.Survival analysisComplications related to PA
Five-year outcomes of coarctoplasty with stent in the paediatric population, results from a retrospective single-centre cohort with centrally-adjudicated outcomes
Authors: Pasebani Y , Rezanejad E , Khalili Y , Zamani R , Ghaemi H R , Jafari F , Moosavi J , Mohebbi B , Abdi A , Sabri M , Montazeri Namin S , Rafati A , Sadeghipour P , Mortezaeian H 
Published: November 2023

Abstract

Background
Stenting is being increasingly used as a therapeutic option for coarctation of aorta (CoA) in paediatric patients. Although the short and mid-term outcomes are well-documented, data on long-term results are scant.

Purpose
The current study aimed to determine the long-term safety and efficacy of aortic coarctation stenting via centrally-adjudicated outcomes.

Methods
The present study is a retrospective cohort conducted at a large tertiary cardiovascular centre. All patients aged up to 15 years who had undergone de novo stenting for CoA between 2006 and 2017 were recruited. Both immediate and 5-year outcomes were investigated. Immediate outcomes, including procedural and in-hospital, were retrieved from electronic medical records, re-analysed, and finally adjudicated by the clinical event committee (CEC). The rates of five-year recoarctation, stent fracture, aneurysmal/pseudoaneurysmal formation, and all-cause mortality were reported as the main long-term outcomes. The first three mentioned outcomes were adjudicated only in patients with available predefined appropriate imaging follow-up tests (Figure 1). Patients who had available recorded 5-year clinical visits and those who answered the phone interview were evaluated for the 5-year mortality.

Results
A total of 274 patients (98 [35.8%] were female with median [interquartile range] age of 9 [6-12] years) who underwent de novo coarctoplasty with stent were included. Procedural success was achieved in 251 (91.6%) of the patients. Immediate complications were observed in a total of 24 (8.75%) as follows: There were 1 (0.3%) stent migration, 3 (1.1%) small, non-expanding non-flow-limiting aortic wall injuries, and 2 (0.7%) ischemic strokes. Also, there were 18 (6.6%) major vascular access complications, the most common of which was acute limb ischemia in 8 (2.9%) patients, followed by 5 (1.8%) pseudoaneurysms, 3 (1.1%) retroperitoneal haemorrhages, 1 (0.3%) arteriovenous fistula, and 1 (0.3%) large hematoma. A total of 4 (1.4%) in-hospital mortality occurred. The 5-year cumulative incidence of stent fracture, recoarctation of the aorta, and aneurysm/pseudoaneurysm of the aorta were 17/100 (17%), 73/154 (48%), and 8/101 (7.92%), respectively. Five-year all-cause mortality occurred in 4/251 (1.6%) (Table 1).

Conclusion
In our study, coarctoplasty with stent was safe and effective in the paediatric population during long-term follow-up. Developmental changes in childhood resulting in stent-size mismatch are suggested as a potential aetiology for a high re-intervention rate. Of note, re-intervention was safe and effective.Figure 1Table 1
Pulmonary vasoconstriction plays a major role in the acute phase of pulmonary embolism in pigs
Authors: Tang Merit V , Emilie Kirk M , Valentin Hansen J , Dam Lyhne M , Dahl Kramer A , Pedersen C , Andersen A , Gammelgaard Schultz J , Karout L , Nielsen-Kudsk J E 
Published: November 2023

Abstract

Background
Pulmonary vascular resistance (PVR) increases in acute pulmonary embolism (PE) due to both mechanical obstruction and pulmonary vasoconstriction. Attenuation of adverse pulmonary vasoconstriction has shown promise as a treatment of acute PE in experimental studies. However, clinical trials have failed to replicate these beneficial effects in PE patients.
Time may be a factor in this discrepancy between experimental and clinical studies. In experimental PE studies, PVR acutely increases and decreases within hours. Meanwhile in clinical trials, the time from symptom onset to study inclusion is often days, at which time PVR may already be decreased. Whether this decrease in PVR is due to reduced mechanical obstruction by endogenous fibrinolysis or attenuation of adverse pulmonary vasoconstriction is unknown.

Purpose
To investigate the contribution of the mechanical obstruction and pulmonary vasoconstriction to PVR in two days following PE in pigs.

Methods
In a controlled animal study, using an estabilished model of autologous PE in pigs (PE, n=12 ∼60 kg), changes in pulmonary hemodynamics and clot burden were evaluated. CT pulmonary angiograms were performed at baseline (BL), after acute PE and the following two days, quantifying clot burden using the Qanadli Obstruction Score. Hemodynamics were evaluated with real-time biventricular catheterization and systemic invasive pressures. To evaluate the vasoactive component, pulmonary vasodilators sildenafil (bolus 0.1 mg/kg) and oxygen (inspired fraction of oxygen 40%) were administered.

Results
Following PE, both mean PVR (Figure 1A) and pulmonary obstruction scores (Figure 1B) increased. PVR decreased at day one and remained unchanged at day two (Figure 1A). Meanwhile, mean obstruction score decreased slightly at day one but remained elevated throughout the study (Figure 1B). Vasodilatation induced by sildenafil and oxygen (SIL+OXY) decreased PVR at day zero but displayed no effect on the subsequent days (Figure 1A).

Conclusion
Pulmonary vasoconstriction and mechanical obstruction both contributed to the increase in PVR during the acute phase of PE. PVR decreased towards levels of baseline already day one and remained unchanged at day two. The contribution of pulmonary vasoconstriction decreased at day one and two, while the mechanical obstruction remained significantly increased. Accordingly, the effects of pulmonary vasodilators were only present in the acute phase of PE.Figure 1
Right ventricular-arterial coupling and exercise-induced tricuspid regurgitation
Authors: Mondellini G M , Rusconi I , Giustiniani A , Novello G , Dinardo G , Serrantoni V , Moscardelli S , Torta D , Losito M , Bursi F , Guazzi M 
Published: November 2023

Abstract

Background
Right Ventricular-pulmonary Artery (RV-PA) coupling is prognostic in a variety of cardiovascular disorders, including heart failure with preserved ejection fraction, pulmonary hypertension and severe aortic stenosis. The functional aspects of RV-PA coupling are less investigated than rest ones especially in what is their interaction with tricuspid regurgitation (TR).

Purpose
We sought to understand the association of RV-PA coupling with extent of exercise induced TR by combining gas exchange abnormalities through cardiopulmonary exercise test (CPET) imaging.

Methods
We prospectively studied patients with indication to perform a CPET imaging test due to dyspnoea and underlying heart disease of any left ventricular ejection fraction. Demographics, baseline characteristics, echo and CPET parameters [(e.g. VE/VCO2 slope and peak oxygen consumption (pVO2)] were evaluated across 3 sub-cohorts phenotyped according to TR severity pattern at rest and at peak exercise . Mitral and tricuspid regurgitation degree were adjudicated accordingly to ESC guidelines. RV-PA coupling function was assessed through the ratio between tricuspid annular plane systolic excursion (TAPSE), and non-invasive pulmonary artery systolic pressure (PASP) with echocardiography. Dynamic TR was defined as detection of at least moderate TR grade from rest to stress.

Results
A total of 73 patients (age 67±13 years, 52% men), with a mean LVEF of 50±14% were studied. Overall 50 (69%) patients had less than moderate TR which did not increase during stress (Controls), 11 (15%) patients exhibited a dynamic TR pattern (Group 1), and 12 (16%) already had significant TR at rest (Group 2). TAPSE/PASP at rest and peak exercise were significantly lower in Group 1 and 2 compared to Controls (Figure), while the mean PAP/cardiac output (CO) ratio at rest and at peak exercise were significantly higher (Table). As to gas exchange data, Group 1 and 2 exhibited a lower median peak VO2, while Group 2 patients had also a higher median VE/VCO2 slope compared to Controls (Table). 10 (14%) patients showed at least moderate mitral regurgitation (significant MR) at rest and the prevalence of significant MR at peak was higher in group 1 and 2 compared to Controls (Table). A weak negative correlation was found between TAPSE/PASP slope and E/e’ ratio at peak exercise (r =-0.435, p&lt;0.01).

Conclusion
In presence of exercise dyspnoea, patients with dynamic TR during maximal exercise exhibit a worse degree of RV-PA coupling and gas exchange profile compared to the group without significant exercise-induced TR. Moreover, the exercise-induced TR profile behaves similarly to the one of those presenting with moderate to severe TR already detectable at rest. Phenotyping dynamic TR and RV-PA warrants further evidence on therapies and outcomes prediction in exertional dyspnoea patients.FigureTable