bluuebunny/crossref_metadata_2025 huggingface dataset:
Neo-aortic dimensions and coronary take-off angle in adolescent and young adult patients with transposition of the great arteries after the arterial switch operation
Authors: Verheijen D , Engele L J , Stoger J L , Mertens B , Koolbergen D R , Hazekamp M G , Jukema J W , Egorova A D , Vliegen H W , Bouma B J , Jongbloed M R M , Kies P
Published: November 2023

Objective

Aim of this study was (1) to assess the relation between neo-aortic dimensions and coronary angle take-off in adult patients with transposition of the great arteries (TGA) late after the arterial switch operation (ASO) and (2) to evaluate if temporal increase in the neo-aortic dimensions is associated with a decrease of the coronary angle take off.

Methods

In this retrospective two-center cohort study, all patients with TGA who underwent ASO and had ≥1 CT-angiography (CTA) of the coronary arteries at the age of ≥ 16 years old were included. Neo-aortic dimensions and coronary angle take-off were measured with CTA, Figure 1.

Results

Eighty-one patients, 69% male and median age 21.0 (18.5-22.8) years, were included. Maximum neo-aortic diameter was 39.2±5.3mm (corrected for BSA 20.9±2.7mm/m2). Sixty-one (76%) patients had a coronary take-off angle of <45° at baseline. No correlation between the maximum neo-aortic diameter and coronary take-off angle of the right coronary artery (RCA) (R=0.161, p=0.150) and left coronary artery (LCA) (R=0.089, p=0.428) was found, Figure 2A-B. During a mean follow up of 7.9±3.1 years in patients with 2 CTAs (n=26), the average increase of maximum neo-aortic diameter at the level of sinus of Valsalva was 0.16±0.20mm/year and a poor correlation between the change in maximum aortic diameter and change in coronary take-off angle of the RCA (R=0.126, p=0.541) and LCA (R=0.311, p=0.158) was observed, Figure 2C-D.

Conclusions

This study reports slightly increased neo-aortic dimensions and high prevalence of acute coronary angle take-off in young adults late after ASO. The average neo-aortic growth rate of 0.16 mm/year at the level of the sinus of Valsalva is twice as fast as reported for healthy adults. However, no significant correlation was found between the neo-aortic dimensions and coronary take-off angle at baseline or during follow-up of almost 8 years in this group.Figure 1Figure 2
Fractional flow reserve in anomalous aortic origin of coronary arteries to evaluate hemodynamic consequences of high risk anatomical features
Authors: Verheijen D , Egorova A D , Jongbloed M R M , Voskuil M , Van Der Kley F , Kies P , Vliegen H W
Published: November 2023

Introduction

In patients with anomalous aortic origin of a coronary artery (AAOCA) fractional flow reserve (FFR) can be performed for further risk stratification of myocardial ischemia and sudden cardiac death. The aim of this study was to evaluate the hemodynamic consequences (accessed with FFR) of the high-risk anatomical features in AAOCA.

Methods

In this prospective cohort study, all consecutive patients with AAOCA in whom diagnostic work-up according to the MuSCAT trial, including FFR, was performed between July 2020 and January 2023 in our tertiary AAOCA referral center, were included. Presence and length of the intramural segment was assessed using the interluminal space (ILS) on the CTA and orifice geometry using IVUS.

Results

Thirty patients, 57% female and mean age at AAOCA diagnosis of 48.7±15.3 years, were included. Twenty-seven (90%) patients presented with an anomalous aortic origin of the right coronary artery. FFR adenosine was measured in all patients (Figure 1), and showed a significant relation with: 1. hypoplasia of the proximal segment on CTA (R=0.400, p=0.029); 2. the ILS at 2mm from the ostium on CTA (R=0.517, p=0.003); 3. the length of the intramural course on CTA (R=0.500, p=0.005); 4. the orifice shape on IVUS (R=0.460, p=0.011) and 5. the minimal lumen area on IVUS (R=0.371, p=0.044).

Conclusions

FFR was significantly reduced in AAOCA with the following high-risk anatomical features: increased hypoplasia of the proximal segment on CTA, lower ILS at 2mm from the ostium on CTA, longer intramural course on CTA, increased slitlike orifice shape on IVUS and lower minimal lumen area on IVUS. These anatomical high-risk features appear to be hemodynamically relevant in the pathophysiology of AAOCA.Figure 1
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

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<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

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

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 <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

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

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<0.001). EPI (OR 0.21, 95% CI: 0.06-0.73, p<0.014), anterior infarct location (OR 17.18, 95 % CI: 2.25-131.05, p<0.006) and LVEF (OR 0.91, 95 % CI 0.86-0.95, p<0.001) were independently associated with LV thrombus. The optimal cut-off value for the prediction of LV thrombus prediction was an EPI <1 (sensitivity 56.3% and specificity 80.4%). 13% (n=18) of patients with an EPI <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 <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

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 (<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

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

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

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<0.001; +0.427, p<0.001; and -0.561, p<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.
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

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

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

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 (<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

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

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<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

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 > 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

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

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