bluuebunny/crossref_metadata_2025_split huggingface dataset:
The impact of tricuspid regurgitation on the timing of pulmonary valve replacement in adult patients after Tetralogy of Fallot repair
Authors: Brancaccio G , Fugallo D , Trezzi M , Bordonaro V , Secinaro A , Piccinelli E , Miraldi F , Butera G , Chinali M , Lisignoli V , Iacobelli R , Leonardi B , Iodice F , Galletti L 
Published: October 2024

Abstract

Background
Tricuspid regurgitation (TR) in patients with repaired tetralogy of Fallot (rTOF) is an important finding with a wide spectrum of etiologies. TR represents an important risk factor in adult patients with rTOF, influencing clinical outcomes and the requirement for additional surgical interventions. In this study, we reviewed the long-term outcomes of TR in ACHD patients with rTOF as well as its impact on the timing of surgical pulmonary valve replacement (PVR).

Methods
This was a retrospective analysis of a single institutional cohort which included 149 ACHD patients with a history of rTOF (transannular patch only) with CMR data, complete follow-up, and a recent (&lt; 1 year) outpatient clinic visit.

Results
Overall follow-up time was 25.5 years (95% CI 24.5,26.4). Between 2002 and 2021, 93 patients required isolated PVR at a mean time from TOF repair of 16.6 years (95% CI 15.5,17.7) and at a mean age of 17.9 years (95% CI 16.8,19.0). In the whole cohort, significant TR was associated with a two-fold increase in the likelihood of requiring PVR (log-rank p = 0-.0023, HR 2.02; 1.09-4-47). Moderate to severe TR was more frequent in those who underwent VSD closure through a right atriotomy (17 versus 0, p = .0002) and affected time to PVR (13.9 versus 16.3 years, p = .0071), with equal preoperative RVEDVi at CMR. After PVR, RVEDVi normalized in all patients, regardless of the preoperative amount of TR (median 115.3 ml/m2 versus 112.3 ml/m2, p = .7). At a median time of 6.5 years from PVR, 13 patients (13/93, 14%) underwent redo PVR.

Conclusions
The preoperative severity of tricuspid regurgitation (TR) may have a substantial role on the likelihood of patients needing PVR at a younger age. This, in turn, could result in a reduced duration of freedom from valve failure. Replacing the pulmonary valve too early can effectively increase the lifetime number of procedures, preserving the function of the tricuspid valve at the time of TOF repair is of paramount importance.
Interaction between right atrial function reserve and liver stiffness after complete repair of tetralogy of fallot: a pilot study
Authors: Chik B W K , Chan K L , Chow P C , Yu C K M , Ho S C S , Cheung Y F 
Published: October 2024

Abstract

Background
Impaired right atrial mechanics is reported in patients with repaired tetralogy of Fallot (TOF) and is associated with liver stiffness. However, whether right atrial functional reserve correlates with liver stiffness is unknown.

Purpose
This study tested the hypothesis that right atrial reserve capacity is limited in patients with repaired TOF and explore its relationships with right ventricular function and liver stiffness.

Methods
10 patients (45% male) aged 19.04 ± 3.76 at 16.70 ± 4.30 years after repair and 15 controls (60% male, aged 20.31 ± 1.25 years) were studied. RA mechanics was assessed by speckle-tracking echocardiography (STE) at rest and during bicycle exercise, with quantification of positive, negative, and total strain, and strain rates at ventricular systole (aSRs), early diastole (aSRed), and atrial contraction (aSRac). Right atrial function reserve (RAFR) is calculated as (∆RA total strain x [1-1/ RA total strain at baseline]). RV systolic and diastolic function was quantified using Doppler interrogation and STE. Hepatic shear wave velocity (c) and tissue elasticity (E) were measured using two-dimensional shear wave elastography.

Results
At rest, patients had significantly lower RA positive, negative and total strain, aSRs and aSRed than control subjects (all P&lt;0.02). Shear wave velocity and hepatic E value were significantly higher in patients than controls (both P&lt;0.02). Compared with controls, patients had significantly lower RA positive, negative and total strain, aSRs, aSRed, and aSRac at exercise (all P&lt;0.05). No significant difference in hepatic shear wave velocity and tissue elasticity were found between patients and control subjects at exercise (all P&gt;0.05). Both baseline liver stiffness indices correlated negatively with RAFR, RA positive and total strain and aSRed at exercise (p&lt;0.05 for all). RV function parameters, including baseline trans-tricuspid early and late diastolic inflow velocity (E, A), systolic, early and late diastolic tricuspid annular velocity (s, e, a), isovolumetric acceleration (IVA), RV systolic and diastolic reserve correlated significantly with RA positive and total strain at exercise (P&lt;0.05 for all).

Conclusions
In young adults with repaired TOF, right atrial function reserve is impaired, and is associated with increased liver stiffness and RV dysfunction.RA mechanics from rest to exerciseStress RA mechanics and liver stiffness
Outcome of pregnancy in women with unrepaired atrial septal defect - study of a single tertiary centre
Authors: Kecskemeti D , Nagy Z S , Szabo B , Pataki S Z , Szegedi M , Temesvari A , Balint O H 
Published: October 2024

Abstract

Background
Patients with unrepaired atrial septal defect (ASD) are prone to develop arrhythmia, right heart failure and to suffer paradoxical embolism. The risk might be even bigger during pregnancy due to the hemodynamic changes combined with the hypercoagulable state.

Purpose
To evaluate maternal, foetal, and neonatal outcome of pregnancy in women with unrepaired ASD.

Methods
A retrospective study of 43 pregnancies in 24 women with unrepaired ASD was performed in our tertiary centre from 2004. Previous maternal cardiovascular events and events during pregnancy (maternal, neonatal, and obstetric) were documented.

Results
24 women with unrepaired ASD (median age: 27.5 ± 6.2) had 43 pregnancies resulting in one termination, 4 miscarriages and 38 live births. 55% were primigravida and 2 twin pregnancies were documented. At baseline 2 women (10.5%) were in NYHA functional class II and signs of right ventricle (RV) volume overload were detected on echocardiography in 18 cases (47%). 2 women had a history of supraventricular tachycardia. Median gestation at delivery was 39 ± 2.6 weeks. No thromboembolic events, pulmonary hypertension, or desaturation occurred during pregnancy. However maternal cardiovascular complications all affected women with RV volume overload: 2 cases (5.2%) of supraventricular tachycardia, 2 (5.2%) cases with NYHA functional class deterioration and 1 case (2.6%) of syncope were documented. Only one obstetric event; preeclampsia occured. Neonatal complications were as follows: premature delivery in 3 cases (7.9%) and recurrence of congenital heart disease (CHD) in 4 pregnancies (10.5%). After the pregnancy device or surgical closure of the ASD was indicated for 11 women (45.8%). Since then, five already underwent device closure, one had surgical repair, while five women are still waiting for intervention.

Conclusions
Our results align with the mWHO classification. Pregnancy with an unrepaired ASD is well tolerated and has a low risk of neonatal and maternal cardiovascular events. However, women with right ventricle volume overload are prone to develop arrhythmia or right heart failure during pregnancy. Therefore, cardiology follow-up during pregnancy should be decided on an individual basis. In our cohort no thromboembolic events were documented during pregnancy, however the prevalence of congenital heart disease in the foetus was higher compared to the literature.
Effect of Fontan circulation on body composition and correlation with exercise capacity
Authors: Pomiato E , Rasoul H , Duong P , Frigiola A 
Published: October 2024

Abstract

Background
There is growing attention to body composition in Fontan patients (FP) and preliminary results suggest that FP have an increased fat mass and a decreased muscle mass compared to the healthy population. FP also have reduced exercise capacity compared to health peers. However, data are still limited.

Purpose
The aim of our study was to compare the body composition (BC) of adult patients with Fontan circulation and healthy controls and to assess the correlation with functional capacity.

Material and Methods
Case control study on adult patients with Fontan circulation (FP) and age and gender matched healthy controls (HC). The following characteristics were recorded: age, gender, morphology of the systemic ventricle, type of Fontan circulation, level of physical activity (PAR, 0-7 points, 0 = sedentary - 7 = running &gt; 3hours/week). S-score muscle, T-score bone, fat mass (FM%), intra-muscular adipose tissue (IMAT), abdominal adipose tissue (AAT), intracellular water (ICW), extracellular water (ECW), total body water (TBW), were measured through multi-frequency bioelectrical impedance technology in the FP and in the HC. Correlation was made for FP between body composition and oxygen consumption at peak exercise (VO2 peak), VE/VCO2 slope and NYHA class as indicator of functional capacity.

Results
Thirty Fontan patients (FP, mean age 24±6 yrs, 70% males) and 30 matched healthy controls (HC) were recruited in the study. The systemic ventricle was dominant left in 14 patients (47%), dominant right in 16 (53%). Four patients had atrio-pulmonary connection, the remaining were palliated with a lateral tunnel (n=26, 87%). The analysed parameters are summarised in Table 1. PAR was reduced in FP compared to HC (median 4.0 points in FP versus 7.0 points in HC, p 0.024). There was no difference in the BMI, fat mass, and body water between FP and HC. However, FP showed significantly lower S and T scores compared to the HC (S-score: -0,75 in FP versus 0,0 in HC, p 0.003. T-score: -0.9 in FP versus -0.25 in HC, p 0.001). In FP, NYHA Class was I in 28 patients (93%), II in 1 (3,5%) and III in 1 (3.5%) patient. The mean VO2 was 23.4±5.4mlO2/kg/min and mean VE/VCO2 slope 32±7. There was a direct correlation between VO2 peak and the PAR (rho 0.443, p 0.044). No correlation was found between the VO2 peak, the VE/VCO2 slope or the NYHA Class and the analysed parameters of body composition.

Conclusion
FP have reduced muscle mass, and bone density compared to HC. In addition to the known reduced efficiency of the cardiac pump during exercise in FP, the lower level of conditioning contributes to the reduced exercise capacity in this population. Programs aiming to encourage physical activity are key and should be part of patients’ care.
Recruitment of CCR5-positive T cells in pulmonary vascular lesions in idiopathic- and connective tissue disease associated- pulmonary arterial hypertension
Authors: Ohta-Ogo K , Ishibashi-Ueda H , Ogo T , Ikeda Y , Inagaki T , Matsumoto M , Amemiya K , Nakaoka Y , Hatakeyama K 
Published: October 2024

Abstract

Background
C-C chemokine receptor type 5 (CCR5) is a chemokine receptor predominantly expressed on leukocytes including T cells, macrophages, and dendritic cells, and is involved in the process by which T cells are attracted to specific tissues and organs. Although CCR5 has been shown to be expressed on macrophages and pulmonary artery smooth muscle cells in lung samples from human idiopathic pulmonary arterial hypertension (PAH) and some animal models, its expression on T cells in human PAH lungs has not been investigated.

Purpose
To investigate the immunohistochemical expression of CCR5 on T cells infiltrating in pulmonary vascular lesions of human PAH tissues, particularly from patients with idiopathic PAH and PAH associated with connective tissue disease (CTD-PAH), where inflammation has been implicated in the pathogenesis.

Methods
Subjects included 25 postmortem cases of idiopathic PAH (14 cases, a mean age of 34 ± 10 years, 8 female cases) and CTD-PAH (11 cases, 48 ± 13 years, all female). We performed immunohistochemistry using antibodies against CCR5 and CD3 (for T cells) on formalin-fixed paraffin-embedded sections. To semi-quantify, we selected the three small muscular pulmonary arteries (80-150 µm in external diameter) with the strongest CCR5-positive cell infiltration in each case, counted the number of positive cells, and the average were shown as the number of cells per vessel.

Results
In all cases, regardless of whether IPAH or CTD-PAH, a subset of infiltrating inflammatory cells into or accumulating around vessels was CCR5-positive. Specifically, CCR5-positive inflammatory cells were found in remodelled pulmonary arteries, such as plexiform lesions and constrictive lesions with intimal thickening and/or medial hypertrophy, as well as perivascular space. The average number of CCR5-positive cells per small muscular pulmonary artery was 8.1/vessel. Where pulmonary venous occlusive lesions were seen, CCR5-positive cells were also present in and around them. The majority of CCR5-positive inflammatory cells appeared to be T cells, based on morphology and CD3 immunopositivity in the serial sections.

Conclusion(s)
Activated T cells showing CCR5 immunopositivity were recruited to remodelled pulmonary vascular lesions in both idiopathic PAH and CTD-PAH. This implicates CCR5-positive T cells as a common key player and that CCR5 signalling is involved in the pathogenesis of PAH in concert with other inflammatory cells, vascular endothelial cells, and pulmonary artery smooth muscle cells.
Epidemiology of acute pulmonary embolism in children and adolescents from nationwide US hospital data, 2016 and 2019
Authors: Wolf S , Valerio L , Kucher N , Konstantinides S , Klok F A , Cannegieter S C , Barco S 
Published: October 2024

Abstract

Background
Data on the epidemiological burden and comorbidities associated with pulmonary embolism (PE) in children and adolescents is sparse.

Purpose
We aimed to describe the burden of acute PE in children by estimating indicators of incidence and mortality as well as the prevalence of key comorbidities in hospitalized children with (vs. without) PE.

Material and methods
We did a patient-level analysis of the United States Kids' Inpatient Database (KID), including patients aged 0 to 19,cumulatively for the years 2016 and 2019. PE was defined by the ICD-10 Code I26. We calculated annualized, nationwide, age-sex-specific incidence rate, mortality rate, case mortality rate, and proportionate mortality rate. Additionally, we studied the prevalence of 120 clinically selected disorders in children with vs. without acute PE.

Results
Acute PE was recorded in 5,738 cases (58.6% female). A total of 244 (4.3%) patients were aged 0, 214 (3.7%) aged 1-4, 206 (3.6%) aged 5-9, 677 (11.8%) aged 10-14, and 4,392 (76.5%) aged 15-19 years. The incidence rate of PE shows a J-shape curve increasing with increasing age. Proportional mortality rate paralleled the incidence rate peaking at 9.5 (95% CI: 7.3-12.3) deaths per 1,000 deaths for girls and 5.0 (4.0-6.2) deaths per 1,000 deaths for boys aged 15-19 years. In contrast, case mortality rate decreased from 33% (95% CI: 24.8-42.4) among girls and 15.9% (95% CI: 10.8-23) among boys in the first year of age to 2.1% (95% CI: 1.6-2.7) among girls and 4.5% (95% CI: 3.6-5.6) among boys aged 15-19. Table 1 presents key age-sex stratified epidemiologic indicators. The highest absolute prevalence difference (APD) between those with vs. without PE was found for venous thrombosis (APD: 31.8%, 95% CI: 30.6-33), followed by metabolic disorders (APD: 15.2%, 95% CI: 14-16.5) and obesity (APD: 14.7%, 95% CI: 13.7-15.8). Figure 1 presents an overview of the APD of clinically selected disorders in patients with vs. without PE.

Conclusion
This comprehensive overview shows that the burden of PE among children and adolescents is considerable, but with considerable variation across age classes and co-occurrence with different specific comorbidities.Table 1.Figure 1.
Diagnosis of chronic thromboembolic pulmonary disease from an intensive pulmonary embolism treatment center
Authors: Sato M , Ikeda N , Hayashi F , Niikura H , Iijima R , Hara H , Nakamura M 
Published: October 2024

Abstract

Background
The diagnosis of Chronic Thromboembolic Pulmonary Disease (CTEPD) following an episode of acute pulmonary embolism (PE) has exhibited inconsistencies across different healthcare institutions, and the issue of underdiagnosis remains unresolved.

Purpose
The aim of this study is to ascertain the incidence of CTEPD following an episode of acute PE through a follow-up conducted at a specialized PE outpatient clinic located within a PE expert center.

Methods and Results
In this retrospective study, a cohort of 106 consecutive patients (mean age: 63.7±17.5 years, female: 51.9%) diagnosed with acute PE between March 2015 and August 2020 at our hospital were included. Patients who were capable of visiting the hospital received follow-up care at an outpatient clinic specializing in PE. The diagnosis of CTEPD was established in cases where patients, regardless of the presence of pulmonary hypertension, exhibited chronic-phase dyspnea and demonstrated perfusion defects on lung perfusion scintigraphy. Furthermore, individuals with a mean pulmonary arterial pressure ≥25 mmHg and a pulmonary capillary wedge pressure ≤15 mmHg, as determined by right heart catheterization, were diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH). During the follow-up period (median duration: 27.5 months), 23 patients were diagnosed with CTEPD, and 10 patients were diagnosed with CTEPH. The cumulative incidence rates of CTEPD and CTEPH were as follows: 20.8% and 10.8% at 1 year, 26.9% and 12.7% at 2 years, respectively; and 28.9% at 3 years for CTEPD. Signs of RV dysfunction on CT or Echocardiography may have contributed to the detection of CTEPH and were discussed with respect to parameters indicating RVdisfunction.

Conclusions
In present study, the cumulative incidence of CTEPD and CTEPH is notably elevated. This results suggest that active surveillance after acute PE follow-up practices may have contributed to an enhanced rate of diagnosis or that chronic PE or CTEPH may have been included at the time of PE diagnosis.Cumulative Incidence of CTEPDCumulative Incidence of CTEPH
TAPSE/sPAP by echocardiography to detect elevated pulmonary vascular resistance&gt; 4 WU in postcapillary pulmonary hypertension patients: insights from the French PH HF registry
Authors: Bauer F , Berthelot E , Eicher J C , Trochu J N , Habib G , Isnard R , Lamblin N 
Published: October 2024

Abstract

Background
Post-capillary pulmonary hypertension (pcPH) lacks effective treatment options. Patients with a PVR &gt; 4 WU, known as combined pcPH (cpcPH), may benefit from investigational drugs like sotatercept. Identifying cpcPH patients suitable for vasodilation therapy in echocardiography labs is critical.

Purpose
To pinpoint an echocardiographic parameter capable of detecting cpcPH patients with a PVR above 4WU, addressing a common challenge in clinical practice.

Methods
In this study, 225 participants from the French PH-HF registry, all diagnosed with heart failure and a preserved ejection fraction, were examined. These patients underwent right heart catheterization and got ultrasound examinations within a 24-hour timeframe. The pulmonary vascular resistance were calculated using previously established methods, and data from standard echocardiography, including assessments of diastolic function, were gathered.

Results
69 patients exhibited a pulmonary vascular resistance (PVR) greater than 4 WU, while 156 patients had a PVR ≤ 4 WU. Age averaged 69±12 years. Mean pulmonary artery pressure, wedge pressure, cardiac output, and pulmonary vascular resistance were 39±9 mm Hg, 22±4 mm Hg, 5.1±1.7 l/min, and 3.5±2.0 WU, respectively. Comparative analysis between patients with PVR ≤ 4 WU and those with PVR &gt; 4 WU revealed similar ejection fraction (EF), left atrial volume index, and E/A ratio by echocardiography (p=0.97, p=0.69, p=0.43, respectively). However, patients with PVR &gt; 4 WU showed elevated E/e’ (p=0.008), higher systolic pulmonary artery pressure (sPAP, p=0.001), but reduced myocardial S and e’ tissue Doppler velocities (p=0.001) and tricuspid annular plane systolic excursion (TAPSE) (p=10^-5). Notably, the relationship between TAPSE/sPAP and PVR &gt; 4 was curvilinear and strong (p=0.001, FIG 1). Using ROC curve analysis, patients with TAPSE/sPAP &lt; 0.38 mm/mmHg were identified to have PVR &gt; 4 WU with a sensitivity and specificity of 68%.

Conclusion
Within the scope of postcapillary pulmonary hypertension, right ventricular arterial coupling is a simple ultrasound parameter potentially effective in identifying individuals exhbiting PVR &gt; 4 WU with a good sensitivityFigure 1:PRV and coupling plotFigure 2:RV coupling ROC
Effects of beta blockers on outcome in patients with PAH with and without comorbid conditions: a Multicenter Prospective Cohort Study (BNP-PL)
Authors: Waligora M , Kurzyna M , Mularek-Kubzdela T , Skoczylas I , Chrzanowski L , Blaszczak P , Jaguszewski M , Kusmierczyk B , Ptaszynska-Kopczynska K , Grzesk G , Mizia-Stec K , Doboszynska A , Peregud-Pogorzelska M , Lewicka E , Kopec G 
Published: October 2024

Abstract

Background
The current guidelines do not recommend the use of beta-blockers (BB) in patients with pulmonary arterial hypertension (PAH) unless necessitated by comorbidities. However, the evidence regarding the role of BB in PAH is contradictory. This study investigates the effects of BB on clinical outcomes in patients newly diagnosed with PAH, stratified by the presence of cardiovascular comorbidities that justify their use.

Methods
We analyzed data from 806 patients newly diagnosed with PAH, who were prospectively enrolled from March 1, 2018, to August 31, 2023, in the multicentre registry, Database of Pulmonary Hypertension in the Polish population (BNP-PL). The primary endpoints were all-cause mortality and a composite of hospitalization due to right heart exacerbation, syncope, or death. The follow-up period for these patients extended until August 31, 2023. Comorbidities in the study group that indicated the need for BB therapy included hypertension, significant arrhythmia, and coronary artery disease. In the Cox regression analysis, the association between BB use and the predefined endpoints was adjusted for age, baseline European Society of Cardiology risk score, and upfront therapy.

Results
Among the 806 patients, BBs were administered to 469 (58.2%) at the time of PAH diagnosis. In the entire cohort, BB therapy was linked to increased all-cause mortality (29.6% vs 21.4%; p=0.005, Fig 1.A) and a higher incidence of the composite endpoint (34.1% vs 22.6%; p&lt;0.0001, Fig 1.B). The Number Needed to Harm (NNH) for BB treatment, regarding mortality and the composite endpoint, were 12.1 and 8.7, respectively. In patients without comorbidities, BB use significantly raised the risk of both all-cause mortality (Hazard Ratio [HR] 1.81, 95% Confidence Interval [CI]: 1.01-3.15) and the composite endpoint (HR 1.85, 95% CI 1.09-3.14), after adjustments for age, baseline three-strata PAH mortality risk, and upfront therapy. However, in patients with at least one comorbidity, BB therapy showed a neutral effect on both outcomes. Across the whole group, the use of nonselective BB was associated with a higher risk of the composite endpoint (HR 1.78, 95% CI 1.13-2.82, p=0.01) compared to selective β1 agents, although this did not extend to all-cause mortality (HR 1.34, 95% CI 0.79-2.25, p=0.28).

Conclusion
BBs, particularly nonselective, pose significant risks in patients with PAH who lack comorbidities that justify their use.