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The prognostic significance of pretransplant N‐terminal pro‐brain (B)‐type natriuretic peptide (NT‐proBNP) level has not been investigated in lung transplant recipients. The electronic files of 173 patients with chronic lung disease who underwent lung transplantation in 2018–2022 at a tertiary medical center were retrospectively reviewed. Right heart catheterization (RHC) and NT‐proBNP determination were performed preoperatively in all cases. Pretransplant demographic, clinical, and laboratory data were compared between posttransplant survivors and nonsurvivors. Correlations of NT‐proBNP values with lung function and RHC parameters and all‐cause mortality were analyzed. NT‐proBNP level correlated positively with mean pulmonary artery pressure (R = 0.51, p < 0.001) and pulmonary vascular resistance (PVR) (R = 0.45, p = 0.0013), and negatively with diffusing lung capacity for carbon monoxide (R = −0.25, p = 0.0017), cardiac index (R = −0.26, p = 0.001), and cardiac output (R = −0.23, p = 0.004). Over a median follow‐up time of 23.22 months, 74 patients died. On univariate analysis, mortality was significantly associated with higher log‐NT‐proBNP (hazard ratio [HR] = 0.54, 95% confidence interval [CI] 1.15–2.05, p = 0.016), older age at transplant registration (HR = 1.033, 95% CI 1.009–1.058, p = 0.0068), higher PVR (HR 1.15, 95% CI 1.07–1.23, p = 0.015), and lower cardiac output (HR = 0.62, 95% CI 0.42–0.92, p = 0.045). On multivariate analysis adjusted for age, sex, and body mass index, mortality significance was maintained only for higher log‐NT‐proBNP (HR = 1.54, 95% CI 1.12–2.11, p = 0.007). Among lung transplant recipients, pretransplant NT‐proBNP levels correlated well with RHC parameters and were strongly associated with posttransplantation mortality. Assessment of NT‐proBNP may improve risk stratification of lung transplant candidates. [ABSTRACT FROM AUTHOR]

Balloon pulmonary angioplasty (BPA) to treat chronic thromboembolic pulmonary hypertension (CTEPH) is generally reserved for distal obstruction precluding pulmonary endarterectomy (PEA) but can be used in patients with proximal disease who are at high surgical risk or refuse surgery. This single‐center retrospective study compared BPA efficacy in patients with proximal versus distal CTEPH. Of the 478 patients, 36 had proximal disease, follow‐up was 11.6 months and mean number of BPA 6. After BPA, PVR, and mean pulmonary artery pressure decreased significantly in the proximal and distal groups (from 6.5 to 4.0 WU and 39 to 31 mmHg and from 7.6 to 3.8 WU and 44 to 31 mmHg, respectively, p < 0.001 for all comparisons). NYHA class also improved significantly in both groups, from 3 to 2, whereas the 6‐min walk distance, cardiac output, and serum NT pro‐BNP showed significant improvements only in the distal group. Thus, when PEA for CTEPH is technically feasible but not performed due to severe comorbidities or patient refusal, BPA can produce significant hemodynamic improvements, albeit less marked than in patients with distal disease. Better patient selection to BPA might improve outcomes in patients with proximal disease who are ineligible for PEA. [ABSTRACT FROM AUTHOR]

In pulmonary hypertension (PH) associated with chronic lung disease (CLD), identifying patients who would benefit from pulmonary vasodilators is a significant clinical challenge because the presence of PH is associated with poorer survival. This study evaluated the severity of pulmonary circulation impairment in patients with CLD‐PH using pulmonary perfusion single‐photon emission computed tomography/computed tomography (SPECT/CT). This single‐center, observational study enrolled patients with CLD‐PH who had a mean pulmonary arterial pressure (PAP) ≥ 25 mmHg, as confirmed by right heart catheterization. The primary outcome was to measure the percentage of pulmonary perfusion defect (%PPD), calculated by dividing the perfusion defect volume from perfusion SPECT images by the lung volume from CT scan images. The secondary outcome was to assess the correlation between %PPD and baseline characteristics. The median %PPD was 52.4% (interquartile range, 42.5%–72.3%) in 22 patients. In multivariate linear regression analysis, both forced vital capacity (β = 0.58, p = 0.008) and mean PAP (β = 0.68, p = 0.001) were significantly correlated with %PPD. In conclusion, significant correlation between mean PAP and %PPD in patients with CLD‐PH was observed. This noninvasive assessment of %PPD may be useful for evaluating the severity of pulmonary circulation impairment in CLD‐PH. [ABSTRACT FROM AUTHOR]

Therapies for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) include balloon pulmonary angioplasty (BPA) and PH‐specific medical therapy. This study compares survival and its predictors before and after the introduction of BPA. BPA was independently associated with survival; however, there was no difference in overall survival between the two cohorts. [ABSTRACT FROM AUTHOR]

Children with pulmonary hypertension (PH) often demonstrate limited exercise capacity. Data support exercise as an effective nonpharmacologic intervention among adults with PH. However, data on exercise training in children and adolescents are limited, and characteristics of the optimal exercise program in pediatric PH have not been identified. Exercise programs may have multiple targets, including muscle deficits which are associated with exercise limitations in both adult and pediatric PH. Wearable accelerometer sensors measure physical activity volume and intensity in the naturalistic setting and can facilitate near continuous data transfer and bidirectional communication between patients and the study team when paired with informatics tools during exercise interventions. To address the knowledge gaps in exercise training in pediatric PH, we designed a prospective, single arm, nonrandomized pilot study to determine feasibility and preliminary estimates of efficacy of a 16‐week home exercise intervention, targeting lower extremity muscle mass and enriched by wearable mobile health technology. The exercIse Training in pulmONary hypertEnsion (iTONE) trial includes (1) semistructured exercise prescriptions tailored to the participant's baseline level of activity and access to resources; (2) interval goal setting fostering self‐efficacy; (3) real time monitoring of activity via wearable devices; (4) a digital platform enabling communication and feedback between participant and study team; (5) multiple avenues to assess participant safety. This pilot intervention will provide information on the digital infrastructure needed to conduct home‐based exercise interventions in PH and will generate important preliminary data on the effect of exercise interventions in youth with chronic cardiorespiratory conditions to power larger studies in the future. [ABSTRACT FROM AUTHOR]

We aimed to describe the clinical characteristics, healthcare resource utilization (HCRU) and costs, health‐related quality of life (HRQoL), and survival for patients with pulmonary arterial hypertension (PAH), stratified by 1‐year mortality risk at diagnosis. Adults diagnosed with PAH at the Sheffield Pulmonary Vascular Disease Unit between 2012 and 2019 were included. Patients were categorized as low, intermediate, or high risk for 1‐year mortality at diagnosis. Demographics, clinical characteristics, comorbidities, HCRU, costs, HRQoL, and survival were analyzed. Overall, 1717 patients were included: 72 (5%) at low risk, 941 (62%) at intermediate risk, and 496 (33%) at high risk. Low‐risk patients had lower HCRU prediagnosis and 1‐year postdiagnosis than intermediate‐ or high‐risk patients. Postdiagnosis, there were significant changes in HCRU, particularly inpatient hospitalizations and accident and emergency (A&E) visits among high‐risk patients. At 3 years postdiagnosis, HCRU for all measures was similar across risk groups. Low‐risk patients had lower EmPHasis‐10 scores (indicating better HRQoL) at diagnosis and at 1‐year follow‐up compared with intermediate‐ and high‐risk patients; only the score in the high‐risk group improved. Median overall survival decreased as risk category increased in analyzed subgroups. Low‐risk status was associated with better 1‐year survival and HRQoL compared with intermediate‐ and high‐risk patients. HCRU decreased in high‐risk patients postdiagnosis, with the most marked reduction in A&E admissions. The pattern of decreased per‐patient inpatient hospitalizations and A&E visits at 3 years postdiagnosis suggests that a diagnosis of PAH helps to decrease HCRU in areas that are key drivers of costs. [ABSTRACT FROM AUTHOR]

Pulmonary hypertension (PH) in newborns and infants is a complex condition associated with several pulmonary, cardiac, and systemic diseases contributing to morbidity and mortality. Thus, accurate and early detection of PH and the classification of its severity is crucial for appropriate and successful management. Using echocardiography, the primary diagnostic tool in pediatrics, human assessment is both time-consuming and expertise-demanding, raising the need for an automated approach. Little effort has been directed towards automatic assessment of PH using echocardiography, and the few proposed methods only focus on binary PH classification on the adult population. In this work, we present an explainable multi-view video-based deep learning approach to predict and classify the severity of PH for a cohort of 270 newborns using echocardiograms. We use spatio-temporal convolutional architectures for the prediction of PH from each view, and aggregate the predictions of the different views using majority voting. Our results show a mean F1-score of 0.84 for severity prediction and 0.92 for binary detection using 10-fold cross-validation and 0.63 for severity prediction and 0.78 for binary detection on the held-out test set. We complement our predictions with saliency maps and show that the learned model focuses on clinically relevant cardiac structures, motivating its usage in clinical practice. To the best of our knowledge, this is the first work for an automated assessment of PH in newborns using echocardiograms. [ABSTRACT FROM AUTHOR]

Background: Progression of non-target lesions (NTLs) after stenting has been reported and is associated with the triggering of an inflammatory response. The perivascular fat attenuation index (FAI) may be used as a novel imaging biomarker for the direct quantification of coronary inflammation. Objectives: To investigate whether FAI values can help identify changes in inflammation status in patients undergoing stent implantation, especially in NTLs. Methods: Patients who underwent pre- and post-stenting coronary computed tomography angiography (CCTA) examination between January 2015 and February 2021 were consecutively enrolled. The pre- and post-stenting FAIs of the full coronary arteries were compared in both the non- and stent-implanted coronary arteries. Moreover, local FAI values were measured and compared between the NTLs and target lesions in the stent implantations. We also compared changes in plaque type and volume in NTLs before and after stenting. Results: A total of 89 patients (mean age 61 years; male 59) were enrolled. The perivascular FAI values in the full coronary arteries decreased after stenting in both the non- and stent-implanted coronary arteries, similar to those in the target lesions. Conversely, the perivascular FAI values in the NTLs increased after stenting (p < 0.05). In addition, the plaque volumes significantly increased in the NTLs after stenting, regardless of whether they were non-calcified, mixed, or calcified (p < 0.05). Conclusion: Perivascular FAI values and plaque volumes increased in the NTLs after stenting. Perivascular FAI can be a promising imaging biomarker for monitoring coronary inflammation after stenting and facilitate long-term monitoring in clinical settings. Clinical relevance statement: Perivascular fat attenuation index, a non-invasive imaging biomarker, may help identify coronary arteries with high inflammation in non-target lesions and facilitate long-term monitoring, potentially providing an opportunity for more targeted treatment. Key Points: • Perivascular fat attenuation index (FAI) values and plaque volumes increased in the non-target lesions (NTLs) after stenting, suggesting potential focal inflammation progression after stenting. However, stenting along with anti-inflammatory treatment ameliorated inflammation in the full coronary arteries. • Perivascular FAI, a non-invasive imaging biomarker, may help identify coronary arteries with high inflammation in NTLs and facilitate long-term monitoring, potentially providing an opportunity for more targeted treatment. [ABSTRACT FROM AUTHOR]

We sought to investigate differential metabolism in patients with systemic sclerosis (SSc) who develop pulmonary arterial hypertension (PAH) versus those who do not, as a method of identifying potential disease biomarkers. In a nested case-control design, serum metabolites were assayed in SSc subjects who developed right heart catheterization-confirmed PAH (n ¼ 22) while under surveillance in a longitudinal cohort from Johns Hopkins, then compared with metabolites assayed in matched SSc patients who did not develop PAH (n ¼ 22). Serum samples were collected at “proximate” (within 12 months) and “distant” (within 1–5 yr) time points relative to PAH diagnosis. Metabolites were identified using liquid chromatography-mass spectroscopy (LC-MS). An LC-MS dataset from SSc subjects with either mildly elevated pulmonary pressures or overt PAH from the University of Michigan was compared. Differentially abundant metabolites were tested as predictors of PAH in two additional validation SSc cohorts. Longchain fatty acid metabolism (LCFA) consistently differed in SSc-PAH versus SSc without PH. LCFA metabolites discriminated SScPAH patients with mildly elevated pressures in the Michigan cohort and predicted SSc-PAH up to 2 yr before clinical diagnosis in the Hopkins cohort. Acylcholines containing LCFA residues and linoleic acid metabolites were most important for discriminating SSc-PAH. Combinations of acylcholines and linoleic acid metabolites provided good discrimination of SSc-PAH across cohorts. Aberrant lipid metabolism is observed throughout the evolution of PAH in SSc. Lipidomic signatures of abnormal LCFA metabolism distinguish SSc-PAH patients from those without PH, including before clinical diagnosis and in mild disease. NEW & NOTEWORTHY Abnormal lipid metabolism is evident across time in the development of SSc-PAH, and dysregulated long-chain fatty acid metabolism predicts overt PAH. [ABSTRACT FROM AUTHOR]

This manuscript represents the official position of the Korean Society of Echocardiography on valvular heart diseases. This position paper focuses on the diagnosis and management of valvular heart diseases with referring to the guidelines recently published by the American College of Cardiology/American Heart Association and the European Society of Cardiology. The committee sought to reflect national data on the topic of valvular heart diseases published to date through a systematic literature search based on validity and relevance. In the part II of this article, we intend to present recommendations for diagnosis and treatment of mitral valve disease and tricuspid valve disease. [ABSTRACT FROM AUTHOR]

Background: Changes in tricuspid valve (TV) function following transvenous lead extraction (TLE) and their impact on long-term survival have not yet been investigated. Methods: From 3633 patients undergoing lead extraction between 2006 and 2021, TV function before and after TLE was evaluated in 2693 patients. Results: After TLE, the TV function remained unchanged in 82.36% of patients, worsened in 9.54%, and improved in 8.10%. Abandoned leads (odds ratio, OR = 1.712; p = 0.044), fibrotic adhesions between leads and TV apparatus (OR = 3.596; p < 0.001), or right ventricular wall (OR = 2.478; p < 0.001) were predisposed to TV worsening. Non-infectious indications for TLE (OR = 1.925; p < 0.001), the severity of tricuspid valve regurgitation (TVR) before TLE (OR = 3.125; p < 0.001), and lead encapsulation (OR = 2.159; p < 0.001) were predictors of improvement in TV function. Although either worsening or improving TV function had no impact on long-term survival in all patients, decreased TVR severity in the subgroup of patients with initial regurgitation grades 3-4 was associated with a better prognosis (hazard ratio, HR = 0.622; p = 0.005). Conclusions: 1. Changes in TV function after TLE were observed in 17.64% of patients. 2. Various factors can predispose to lead-related TV changes, although the common denominator in these events is an extensive buildup of scar tissue. 3. Worsening TV function had no impact on survival after TLE. In patients with severe TV dysfunction, reduction in TVR following TLE was associated with a 40% reduction in mortality during a mean follow-up of 1673 days. [ABSTRACT FROM AUTHOR]

Heart failure (HF) with preserved ejection fraction (HFpEF) is the most common cause of pulmonary hypertension (PH) worldwide and is strongly associated with adverse clinical outcomes. The American Heart Association recently highlighted a call to action regarding the distinct lack of evidence-based treatments for PH due to poorly understood pathophysiology of PH attributable to HFpEF (PH-HFpEF). Prior studies have described cardiophysiological mechanisms to explain the development of isolated postcapillary PH (ipc-PH); however, the consequent increase in pulmonary vascular (PV) resistance (PVR) may lead to the less understood and more fatal combined pre- and postcapillary PH (cpc-PH). Metabolic disease and inflammatory dysregulation have been suggested to predispose PH, yet the molecular mechanisms are unknown. Although PH-HFpEF has been studied to partly share vasoactive neurohormonal mediators with primary pulmonary arterial hypertension (PAH), clinical trials that have targeted these pathways have been unsuccessful. The increased mortality of patients with PH-HFpEF necessitates further study into viable mechanistic targets involved in disease progression. We aim to summarize the current pathophysiological and clinical understanding of PH-HFpEF, highlight the role of known molecular mechanisms in the progression of PV disease, and introduce a novel concept that lipid metabolism may be attenuating and propagating PH-HFpEF. [ABSTRACT FROM AUTHOR]

Aims: Ischaemic mitral regurgitation (MR) is a dynamic condition influenced by global and regional left ventricular remodelling as well as mitral valvular deformation. Exercise testing plays a substantial role in assessing the haemodynamic relevance of MR and is recommended by current guidelines. We aimed to assess the prevalence, haemodynamic consequences, and prognostic impact of dynamic MR using isometric handgrip exercise., Methods and Results: Heart failure patients with ischaemic cardiomyopathy and at least mild MR who underwent handgrip echocardiography at the University Hospital Duesseldorf between January 2018 and September 2021 were enrolled. Patients were followed-up for 1 year to assess a combined endpoint including all-cause mortality, heart failure hospitalization, mitral valve surgery/interventions, ventricular assist device implantation and heart transplantation. One hundred thirty-three patients with ischaemic cardiomyopathy were included (age 75 ± 10 years; 21% female; LVEF 35 ± 9%). At rest, 70 patients (53%) presented with mild MR, 54 patients had moderate MR (41%), and 9 patients (7%) showed severe MR. Twenty-five patients (20%) with non-severe MR at rest, developed severe MR during handgrip exercise. Patients with dynamic MR had larger left atrial dimensions, increased wall motion score index and larger tenting area at rest. Multivariate analysis identified MR severity during exercise [HR 1.998 (1.367-2.938)] and exercise TAPSE [HR 0.913 (0.853-0.973)] as predictors of the combined endpoint., Conclusions: The haemodynamic changes provoked by isometric exercise unmasked dynamic severe MR in a significant number of patients with non-severe MR at rest. These data may have implications for therapeutic decision-making in symptomatic patients with non-severe MR at rest., (© 2025 The Author(s). ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Aims: To evaluate the association between transcatheter edge-to-edge repair (TEER) and outcomes in patients with significant mitral regurgitation (MR) following acute myocardial infarction (MI), focusing on the aetiology of acute post-MI MR in high-risk surgical patients., Methods and Results: The International Registry of MitraClip in Acute Mitral Regurgitation following Acute Myocardial Infarction (IREMMI) includes 187 patients with severe MR post-MI managed with TEER. Of these, 176 were included in the analysis, 23 (13%) patients had acute papillary muscle rupture (PMR) and 153 (87%) acute secondary MR. The mean age was 70 ± 10 years and 41% were female. PMR patients had fewer cardiovascular risk factors: hypertension (52% vs. 73%, p = 0.04), diabetes (26% vs. 48%, p < 0.01) but a higher left ventricular ejection fraction (45±  15% vs.35±  10%, p < 0.01) compared secondary MR patients. PMR patients were more likely to present in cardiogenic shock (91% vs. 51%, p = 0.001), require mechanical circulatory support (74% vs. 34%, p = 0.01), and had a higher EuroSCORE II (23±  13% vs. 13±  11%, p = 0.011). The median time from MI to TEER was shorter in PMR (6 days) versus secondary MR (20 days) (p < 0.01). Procedural success was similar (87% vs. 92%, p = 0.49) with comparable MR grade reduction. However, PMR patients had significantly higher in-hospital mortality rates (adjusted odds ratio [OR] 3.05, 95% confidence interval [CI] 1.15-8.12, p = 0.02), 30-day mortality rates (unadjusted OR 3.99, 95% CI 1.42-11.26, p = 0.01) and a higher rate of conversion to surgical mitral valve replacement (22% vs. 3%, p < 0.01) (unadjusted OR 8.17, 95% CI 2.15-30.96, p < 0.001). Aetiology of MR, cardiogenic shock, and procedure timing significantly impacted in-hospital mortality. After adjusting for EuroSCORE II and cardiogenic shock, MR aetiology remained the strongest predictor (adjusted OR 6.71; 95% CI 2.06-21.86, p < 0.01)., Conclusion: Transcatheter edge-to-edge repair may be considered a salvage or bridge procedure in decompensated post-MI MR patients of both aetiologies; however, patients with PMR have a higher risk of mortality and conversion to surgery., (© 2025 European Society of Cardiology.)

Background: Four scores have been published in 2022 for assessing mortality risk of patients with tricuspid regurgitation (TR): the TRI-SCORE, those reported by Hochstadt and Wang and the TRIO score. Our objective was to perform an external validation of available scores for predicting mortality and the combined endpoint of mortality and heart failure (HF) admission, in an independent cohort of patients with severe TR and to compare their discriminative ability., Methods: Discriminative ability of the scores for predicting events was assessed by means of receiver operating characteristics (ROC) curves., Results: The validation cohort retrospectively included 614 consecutive patients (69 ± 13 years, 72% women) with severe TR studied with echocardiography in a tertiary care hospital and followed for up to 14 years (median 5 years, p25-75 2-7 years), with 358 deaths and 620 HF admissions on follow-up. Discriminative abilities for predicting death (C-statistic .72 [95% CI .68-.76] for the TRI-SCORE; .75 [.71-.78] for the Hochstadt score; .72 [.68-.76] for the Wang score; and .74 [.70-.78] for the TRIO score, p < .0005 for all) or the combined endpoint (C-statistic .74 [.70-.78]; .74 [.70-.78], .73 [.69-.77] and .76 [.72-.80], respectively, p < .0005 for all) on follow-up were statistically significant for all of them. Paired comparisons among them for predicting both endpoints were all non-significant., Conclusions: All tested scores showed significant and similar discriminative ability for predicting the combined endpoint of mortality or HF admission in this independent validation study of patients with severe TR., (© 2025 Stichting European Society for Clinical Investigation Journal Foundation. Published by John Wiley & Sons Ltd.)

Background: Severe tricuspid regurgitation (TR) is associated with high morbidity and mortality. Isolated TR, defined as TR without overt heart disease, is typical and offers limited cardiac treatment options other than interventional repair or replacement. Survival history of cancer or active cancer treatment may lead to an unnecessary delay of TR treatment., Methods: We included all patients diagnosed with severe TR at the Medical University of Vienna between 2003 and 2016 who had normal left ventricular function and no other valvular lesions. Outcome analysis was performed on cancer type, status and the number of organs affected by cancer., Results: A total of 973 patients were included. 182 (19%) patients had cancer, 52 were active and 130 had a history of cancer at the time of TR diagnosis. Oncologic patients were divided into subgroups of gastrointestinal, skin, glands, gynaecological, breast, urogenital, lung and other cancers. Ten-year mortality of patients with cancer was higher than those without cancer (p < 0.001). Multivariate analysis adjusting for age did not reveal significantly higher mortality in patients with a history of cancer compared to patients without cancer (p = 0.59). Patients with lung, active, or multi-organ cancer showed the highest mortality., Conclusions: Mortality in patients with severe isolated TR is high and increased by active or multi-organ cancer but not by a history of cancer. These patients should be discussed in interdisciplinary cardio-oncology teams to avoid delaying life-saving treatment of TR and cancer., (© 2024 The Author(s). European Journal of Clinical Investigation published by John Wiley & Sons Ltd on behalf of Stichting European Society for Clinical Investigation Journal Foundation.)

Aims: In patients with degenerative mitral regurgitation (DMR), left ventricular (LV) dysfunction is associated with increased risk of heart failure and excess mortality. LV end-systolic diameter (LVESD) is an established trigger for intervention, yet recommended LVESD thresholds apply poorly to patients with small body size. Whether LV normalization to body surface area (BSA) may be used as a trigger for DMR correction is unknown. We examined the link between LVESD index (LVESDi) and outcome in DMR to identify appropriate thresholds for excess mortality., Methods and Results: This study focuses on 2753 consecutive patients with DMR due to flail leaflets diagnosed in tertiary centres from Europe and the United States, with prospective echocardiographic measurement of LVESD and BSA and long-term follow-up. The primary endpoint was mortality after diagnosis under conservative management. Secondary endpoints were mortality under conservative and surgical management and postoperative mortality of patients who underwent surgery. The optimal LVESDi cut-off for mortality prediction was 20 mm/m 2 . Irrespective of management type, 10-year survival was lower with LVESDi ≥20 mm/m 2 than with LVESDi <20 mm/m 2 (both p < 0.001). After covariate adjustment, LVESDi ≥20 mm/m 2 was independently predictive of mortality under conservative management (adjusted hazard ratio [HR] 1.41, 95% confidence interval [CI] 1.15-1.75), and with conservative and surgical management (adjusted HR 1.34, 95% CI 1.17-1.54). LVESDi remained associated with poorer postoperative outcome in patients who underwent intervention. LVESDi showed higher incremental predictive value over the baseline model compared to LVESD. The association between LVESDi ≥20 mm/m 2 and outcome was consistent in subgroups of patients with DMR., Conclusions: In severe DMR due to flail leaflets, LVESDi is a marker of risk additive and incremental to LVESD. Its use in clinical practice should lead to earlier referral to mitral valve surgery and improved long-term outcome., (© 2024 European Society of Cardiology.)

Aims With the 2022 pulmonary hypertension (PH) definition, the mean pulmonary artery pressure (mPAP) threshold for any PH was lowered from ≥25 to >20 mmHg, and the pulmonary vascular resistance (PVR) value to differentiate between isolated post-capillary PH (IpcPH) and combined pre- and post-capillary PH (CpcPH) was reduced from >3 Wood units (WU) to >2 WU. We assessed the impact of this change in the PH definition in aortic stenosis (AS) patients undergoing aortic valve replacement (AVR). Methods and results Severe AS patients (n = 503) undergoing pre-AVR cardiac heart catheterization were classified according to both the 2015 and 2022 definitions. The post-AVR mortality [median follow-up 1348 (interquartile range 948–1885) days] was assessed. According to the 2015 definition, 219 (44% of the entire population) patients had PH: 63 (29%) CpcPH, 125 (57%) IpcPH, and 31 (14%) pre-capillary PH. According to the 2022 definition, 321 (+47%) patients were diagnosed with PH, and 156 patients (31%) were re-classified: 26 patients from no PH to IpcPH, 38 from no PH to pre-capillary PH, 38 from no PH to unclassified PH, 4 from pre-capillary PH to unclassified PH, and 50 from IpcPH to CpcPH (CpcPH: +79%). With both definitions, only the CpcPH patients displayed increased mortality (hazard ratios ≈ 4). Among the PH-defining haemodynamic components, PVR was the strongest predictor of death. Conclusion In severe AS, the application of the 2022 PH definition results in a substantially higher number of patients with any PH as well as CpcPH. With either definition, CpcPH patients have a significantly increased post-AVR mortality. [ABSTRACT FROM AUTHOR]

Tricuspid regurgitation, once considered a relatively benign condition, has now gathered significant attention due to new evidence showing its impact on both short- and long-term follow-up. While surgical intervention remains the established standard approach for treating severe tricuspid regurgitation, current guidelines provide Class I indication for intervention in only a limited set of scenarios. This review delves into the present and future perspectives of surgical tricuspid regurgitation management, examining aspects such as disease prognosis, surgical indications, outcomes, and a comprehensive overview of past and upcoming clinical trials. [ABSTRACT FROM AUTHOR]

Advances in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) over the past decade changed the disease landscape, yet global insight on clinical practices remains limited. The CTEPH global cross‐sectional scientific survey (CLARITY) aimed to gather information on the current diagnosis, treatment, and management of CTEPH and to identify unmet medical needs. This paper focuses on the treatment and management of CTEPH patients. The survey was circulated to hospital‐based medical specialists through Scientific Societies and other medical organizations from September 2021 to May 2022. The majority of the 212 respondents involved in the treatment of CTEPH were from centers performing up to 50 pulmonary endarterectomy (PEA) and/or balloon pulmonary angioplasty (BPA) procedures per year. Variation was observed in the reported proportion of patients deemed eligible for PEA/BPA, as well as those that underwent the procedures, including multimodal treatment and subsequent follow‐up practices. Prescription of pulmonary arterial hypertension‐specific therapy was reported for a variable proportion of patients in the preoperative setting and in most nonoperable patients. Reported use of vitamin K antagonists and direct oral anticoagulants was similar (86% vs. 82%) but driven by different factors. This study presents heterogeneity in treatment approaches for CTEPH, which may be attributed to center‐specific experience and region‐specific barriers to care, highlighting the need for new clinical and cohort studies, comprehensive clinical guidelines, and continued education. [ABSTRACT FROM AUTHOR]

Data on demographic characteristics and therapeutic approaches in Latin American pulmonary arterial hypertension (PAH) patients are scarce. Pulmonary Hypertension Mexican registry (REMEHIP) is a multicenter Mexican registry of adult and pediatric patients, including prevalent and incident cases. Objective: assess clinical characteristics, treatment trends, and in‐hospital outcomes. Inclusion: age >2 years, diagnosis of pulmonary hypertension (PH) (groups 1 and 4), right heart catheterization with mPAP ≥25 mmHg, PWP ≤ 15 mmHg, and PVR > 3 Wood unit (WU). We included 875 PH patients, 619 adults, 133 pediatric idiopathic PAH (IPAH), and 123 chronic thromboembolic pulmonary hypertension (CTEPH) patients. We enrolled 48.4% of the incident and 51.6% of the prevalent adult and pediatric patients. PAH adults: age 43 ± 15, females 81.9%, functional class (FC) (I/II) 66.5%, 6‐min walk distance (6MWD) 378 ± 112 m, mPAP 57.3 ± 19.0 mmHg, confidence interval (CI) 3.3 ± 1.5 L/min/m2, PVR 12.0 ± 8.1 WU. PAH pediatrics: age 9 ± 5, females 51.1%, FC (I/II) 85.5%, 6MWD 376 ± 103 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 16.4 ± 13.5 WU. CTEPH: age 44 ± 17, females 56.1%, FC (I/II) 65.5%, 6MWD 369 ± 126 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 10.5 + 6.5 WU. When we analyzed the IPAH group separately, it sustained a high functional class I/II incidence. REMEHIP shows better functional class in young females with severe PAH than in American and European patients. Also, PAH pediatric patients had a better functional class than other registries. However, our registry also shows that our population's access to specific pharmacologic treatments is still far from optimal. [ABSTRACT FROM AUTHOR]

This retrospective study was conducted to evaluate all‐cause healthcare resource utilization (HCRU) and costs in commercially insured patients living with pulmonary arterial hypertension (PAH) and explore end‐of‐life (EOL)‐related HCRU and costs. Data from the IQVIA PharMetrics® Plus database (October 2014 to May 2020) were analyzed to identify adults (≥18 years) with PAH (PAH cohort) and those without PH (non‐PH cohort). Patients were required to have data for ≥12 months before (baseline) and ≥6 months after (follow‐up) the first observed PH diagnosis (index date) for PAH cohort or pseudo index date for non‐PH cohort. A PAH EOL cohort was similarly constructed using a broader data window (October 2014 to March 2022) and ≥1 month of follow‐up. Annualized all‐cause HCRU and costs during follow‐up were compared between PAH and non‐PH cohorts after 1:1 matching on propensity scores derived from patient characteristics. EOL‐related HCRU and costs were explored within 30 days and 6 months before the death date and estimated by a claims‐based algorithm in PAH EOL cohort. The annual all‐cause total ($183,616 vs. $20,212) and pharmacy ($115,926 vs. $7862; both p < 0.001) costs were 8 and 14 times higher, respectively, in the PAH cohort versus matched non‐PH cohort (N = 386 for each). In PAH EOL cohort (N = 28), the mean EOL‐related costs were $48,846 and $167,524 per patient within 30 days and 6 months before the estimated death, respectively. Hospitalizations contributed 58.8%–70.8% of the EOL‐related costs. The study findings indicate substantial HCRU and costs for PAH. While pharmacy costs were one of the major sources, hospitalization was the primary driver for EOL‐related costs. [ABSTRACT FROM AUTHOR]

Surgical indications for patients with pulmonary arterial hypertension (PAH) and congenital heart defects are controversial. The treat and repair strategy has demonstrated efficacy in adult populations, but there have been no studies on pediatric patients. This study included pediatric patients with PAH and simple congenital heart defects who underwent corrective repair between 2012 and 2021. According to the preoperative treatment strategies, the patients were divided into a regular strategy group (Group 1) and a treat‐and‐repair strategy group (Group 2). Postoperative recovery and follow‐up results were compared between the two groups. A total of 33 patients were included in this study. Group 1 consisted of 19 patients, whereas Group 2 consisted of 14 patients. The pulmonary vascular resistance index in Group 2 was higher than that in Group 1 (10.9 ± 4.1 vs. 8.2 ± 1.6 WU, p = 0.031). There were no differences in postoperative recovery between the two groups (p > 0.05). During follow‐up, five patients were lost (three in Group 1 and two in Group 2). The median follow‐up period was 59 months. One patient died in Group 1, and two patients died in Group 2. There was no significant difference in the survival curve (p = 0.39). At the last follow‐up, another seven patients had experienced a non‐low‐risk condition, with a total of three non‐low‐risk patients in Group 1 and seven in Group 2, including one patient in each group who had a history of ICU admission. According to the ROC curve, a preoperative PVRi <8.2 WU×m2 can predict postoperative persistent low‐risk state, PVRi <5.2 WU×m2 can avoid postoperative death and/or ICU administration. In pediatric patients with PAH and simple congenital heart defects, the treat and repair strategies may provide surgery opportunities, PVRi should be <8 WU×m2, and <5.2 WU×m2 is the best choice. [ABSTRACT FROM AUTHOR]

Chronic thromboembolic pulmonary hypertension (CTEPH) is a sequela of a pulmonary embolus that occurs in approximately 1%−3% of patients. Pulmonary thromboendoarterectomy (PTE) can be a curative procedure, but balloon pulmonary angioplasty (BPA) has emerged as an option for poor surgical candidates. We used the National Inpatient Sample to query patients who underwent PTE or BPA between 2012 and 2019 with CTEPH. The primary outcome was a composite of in‐hospital mortality, myocardial infarction, stroke, tracheostomy, and prolonged mechanical ventilation. Outcomes were compared between low‐ and high‐volume centers, defined as 5 and 10 procedures per year for BPA and PTE, respectively. During our study period, 870 BPA and 2395 PTE were performed. There was a 328% relative increase in the number of PTE performed during the study period. Adverse events for BPA were rare. There was an increase in the primary composite outcome for low‐volume centers compared to high‐volume centers for PTE (24.4% vs. 12.1%, p = 0.003). Patients with hospitalizations for PTE in low‐volume centers were more likely to have prolonged mechanical ventilation (20.0%% vs. 7.2%, p < 0.001) and tracheostomy (7.8% vs. 2.6%, p = 0.017). In summary, PTE rates have been rising over the past 10 years, while BPA rates have remained stable. While adverse outcomes are rare for BPA, patients with hospitalizations at low‐volume centers for PTE were more likely to have adverse outcomes. For patients undergoing treatment of CTEPH with BPA or PTE, referral to high‐volume centers with multidisciplinary teams should be encouraged for optimal outcomes. [ABSTRACT FROM AUTHOR]

Identification of long‐term calcium channel blocker (CCB) responders with acute vasodilator challenge is critical in the evaluation of patients with pulmonary arterial hypertension. Currently there is no standardized approach for use of supplemental oxygen during acute vasodilator challenge. In this retrospective analysis of patients identified as acute vasoresponders, treated with CCBs, all patients had hemodynamic measurements in three steps: (1) at baseline; (2) with 100% fractional inspired oxygen; and (3) with 100% fractional inspired oxygen plus inhaled nitric oxide (iNO). Those meeting the definition of acute vasoresponsiveness only after first normalizing for the effects of oxygen in step 2 were labeled "iNO Responders." Those who met the definition of acute vasoresponsiveness from a combination of the effects of 100% FiO2 and iNO were labeled "oxygen responders." Survival, hospitalization for decompensated right heart failure, duration of CCB monotherapy, and functional data were collected. iNO responders, when compared to oxygen responders, had superior survival (100% vs. 50.1% 5‐year survival, respectively), fewer hospitalizations for acute decompensated right heart failure (0% vs. 30.4% at 1 year, respectively), longer duration of CCB monotherapy (80% vs. 52% at 1 year, respectively), and superior 6‐min walk distance. Current guidelines for acute vasodilator testing do not standardize oxygen coadministration with iNO. This study demonstrates that adjusting for the effects of supplemental oxygen before assessing for acute vasoresponsiveness identifies a cohort with superior functional status, tolerance of CCB monotherapy, and survival while on long‐term CCB therapy. [ABSTRACT FROM AUTHOR]

Echocardiography is a widely used modality for the assessment of right ventricular (RV) function; however, few studies have comprehensively compared the accuracy of echocardiographic parameters using invasively obtained reference values. Therefore, this exploratory study aimed to compare the accuracy of echocardiographic parameters of RV function and RV–pulmonary artery (PA) coupling. We calculated four indices of RV function (end‐systolic elastance [Ees] for systolic function [contractility], τ for relaxation, and β and end‐diastolic elastance [Eed] for stiffness), and an index of RV–PA coupling (Ees/arterial elastance [Ea]), using pressure catheterization, cardiac magnetic resonance imaging, and a single‐beat method. We then compared the correlations of RV indices with echocardiographic parameters. In 63 participants (54 with pulmonary hypertension (PH) and nine without PH), Ees and τ correlated with several echocardiographic parameters, such as RV diameter and area, but the correlations were moderate (|correlation coefficients (ρ)| < 0.5 for all parameters). The correlations of β and Eed with echocardiographic parameters were weak, with |ρ| < 0.4. In contrast, Ees/Ea closely correlated with RV free wall longitudinal strain (RVFW‐LS)/estimated systolic PA pressure (eSPAP) (ρ = −0.72). Ees/Ea also correlated with tricuspid annular plane systolic excursion/eSPAP, RV diameter, and RV end‐systolic area, with |ρ | >0.65. In addition, RVFW‐LS/eSPAP yielded high sensitivity (0.84) and specificity (0.75) for detecting reduced Ees/Ea. The present study indicated a limited accuracy of echocardiographic parameters in assessing RV systolic and diastolic function. In contrast to RV function, they showed high accuracy for assessing RV–PA coupling, with RVFW‐LS/eSPAP exhibiting the highest accuracy. [ABSTRACT FROM AUTHOR]

Current predictors of clinical outcomes after pulmonary thromboendarterectomy (PTE) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) are largely limited to preoperative clinical characteristics. N‐terminal‐pro‐brain natriuretic peptide (NT‐pro‐BNP), a biomarker of right ventricular dysfunction, has not yet been well described as one such predictor. From 2017 to 2021, 816 patients with CTEPH referred to the University of California, San Diego for PTE were reviewed for differences in NT‐pro‐BNP to predict preoperative characteristics and postoperative outcomes up to 30 days post‐PTE. For analysis, NT‐pro‐BNP was dichotomized to less than/equal to or greater than 1000 pg/mL based on the mean of the study population. Mean NT‐pro‐BNP was 1095.9 ±1783.4 pg/mL and median was 402.5 pg/mL (interquartile range: 119.5–1410.8). Of the 816 patients included, 250 had NT‐pro‐BNP > 1000 pg/mL. Those with NT‐pro‐BNP > 1000 pg/mL were significantly more likely to have worse preoperative functional class (III–IV) and worse preoperative hemodynamics. Patients with NT‐pro‐BNP > 1000 pg/mL also tended to have more postoperative complications including reperfusion pulmonary edema (22% vs. 5.1%, p < 0.001), airway hemorrhage (8.4% vs. 4.9%, p = 0.075), residual pulmonary hypertension (11.9% vs. 3.1%, p < 0.001), and 30‐day mortality (4.8% vs. 1.1%, p = 0.001). Even after adjusting for confounders, patients with NT‐pro‐BNP > 1000 pg/mL had a 2.48 times higher odds (95% confidence interval: 1.45–4.00) of reaching a combined endpoint that included the above complications. Preoperative NT‐pro‐BNP > 1000 pg/mL is a strong predictor of more severe preoperative hemodynamics and identifies patients at higher risk for postoperative complications. [ABSTRACT FROM AUTHOR]

Pulmonary arterial hypertension (PAH) is driven by pathologies associated with increased metabolism such as pulmonary revascularization, vasoconstriction and smooth muscle cell proliferation in pulmonary artery wall. 18‐fluorodeoxyglucose positron emission tomography (18FDG‐PET) is an imaging technique sensitive to glucose metabolism and might be considered as a non‐invasive method for diagnosis due to significant role of inflammation in idiopathic pulmonary artery hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH). The present study aimed to investigate the role of PET/CT imaging of patients with IPAH and CTEPH as an alternative diagnosis method. Demographic characteristics, FDG uptake in lungs, pulmonary artery and right ventricle (RV) of 17 patients (10 IPAH, 7 CTEPH), and 30 controls were evaluated. PET scanning, 6‐min walk test, pro‐BNP level, right heart catheterization of patients were performed both at the onsert and after 6‐month PAH specific treatment. IPAH and CTEPH patients had significantly higher left lung FDG (p = 0.006), right lung FDG (p = 0.004), right atrial (RA) FDG (p < 0.001) and RV FDG (p < 0.001) uptakes than controls. Positive correlation was detected between the RV FDG uptake and the mean pulmonary artery pressure (mPAP) (r = 0.7, p = 0.012) and between the RA FDG uptake and the right atrial pressure (RAP) (r = 0.5, p = 0.02). Increased RV FDG and RA FDG uptakes predicts the presence of pulmonary hypertension and correlates with mPAP and RAP, respectively, which are important indicators in the prognosis of PAH. Further studies are required whether FDG PET imaging can be used to diagnose or predict the prognosis of pulmonary hypertension. [ABSTRACT FROM AUTHOR]

The optimal management of heart valve disease (HVD) is still debated and many studies are underway to identify the best time to refer patients for the most appropriate treatment strategy (either conservative, surgical or transcatheter interventions). Exercise pulmonary hypertension (PH) can be detected during exercise stress echocardiography (ESE) and has been demonstrated to have an important prognostic role in HVD, by predicting symptoms and mortality. This review article aims to provide an overview on the prognostic role of exercise PH in valvulopathies, and its possible role in the diagnostic-therapeutic algorithm for the management of HVD. [ABSTRACT FROM AUTHOR]

Background: Alzheimer's disease (AD) is a chronic neurodegenerative disease needing effective therapeutics urgently. Sildenafil, one of the approved phosphodiesterase-5 inhibitors, has been implicated as having potential effect in AD. Objective: To investigate the potential therapeutic benefit of sildenafil on AD. Methods: We performed real-world patient data analysis using the MarketScan® Medicare Supplemental and the Clinformatics® databases. We conducted propensity score-stratified analyses after adjusting confounding factors (i.e., sex, age, race, and comorbidities). We used both familial and sporadic AD patient induced pluripotent stem cells (iPSC) derived neurons to evaluate the sildenafil's mechanism-of-action. Results: We showed that sildenafil usage is associated with reduced likelihood of AD across four new drug compactor cohorts, including bumetanide, furosemide, spironolactone, and nifedipine. For instance, sildenafil usage is associated with a 54% reduced incidence of AD in MarketScan® (hazard ratio [HR] = 0.46, 95% CI 0.32– 0.66) and a 30% reduced prevalence of AD in Clinformatics® (HR = 0.70, 95% CI 0.49– 1.00) compared to spironolactone. We found that sildenafil treatment reduced tau hyperphosphorylation (pTau181 and pTau205) in a dose-dependent manner in both familial and sporadic AD patient iPSC-derived neurons. RNA-sequencing data analysis of sildenafil-treated AD patient iPSC-derived neurons reveals that sildenafil specifically target AD related genes and pathobiological pathways, mechanistically supporting the beneficial effect of sildenafil in AD. Conclusions: These real-world patient data validation and mechanistic observations from patient iPSC-derived neurons further suggested that sildenafil is a potential repurposable drug for AD. Yet, randomized clinical trials are warranted to validate the causal treatment effects of sildenafil in AD. [ABSTRACT FROM AUTHOR]

The use of left ventricular assist devices (LVAD) has significantly increased in the last years, trying to offer a therapeutic alternative to heart transplantation, in light also to the significant heart donor shortage compared to the growing advanced heart failure population. Despite technological improvements in the devices, LVAD-related mortality is still fairly high, with right heart failure being one of the predominant predictors. Therefore, many efforts have been made toward a thorough right ventricular (RV) evaluation prior to LVAD implant, considering clinical, laboratory, echocardiographic, and invasive hemodynamic parameters. However, there is high heterogeneity regarding both which predictor is the strongest as well as the relative cut-off values, and a consensus has not been reached yet, increasing the risk of facing patients in which the distinction between good or poor RV function cannot be surely reached. In parallel, due to technological development and availability of mechanical circulatory support of the RV, LVADs are being considered even in patients with suboptimal RV function. The aim of our review is to analyze the current evidence regarding the role of RV function prior to LVAD and its evaluation, pointing out the extreme variability in parameters that are currently assessed and future prospective regarding new diagnostic tools. Finally, we attempt to gather the available information on the therapeutic strategies to use in the peri-operative phase, in order to reduce the incidence of RV failure, especially in patients in which the preoperative evaluation highlighted some conflicting results with regard to ventricular function. [ABSTRACT FROM AUTHOR]

Aims: Current evidence on the prognostic value of exercise stress echocardiography (ESE) in asymptomatic patients with low-gradient severe aortic stenosis (AS) is limited. Therefore, this study aimed to elucidate its prognostic implications for patients with low-gradient severe AS and determine the added value of ESE in risk stratification for this population. Methods and results: This retrospective observational study included 122 consecutive asymptomatic patients with either moderate [mean pressure gradient (MPG) < 40 mmHg and aortic valve area (AVA) 1.0-1.5 cm²] or low-gradient severe (MPG < 40 mmHg and AVA < 1.0 cm²) AS and preserved left ventricular ejection fraction (≥50%) who underwent ESE. All patients were followed up for AS-related events. Of 143 patients, 21 who met any exclusion criteria, including early interventions, were excluded, and 122 conservatively managed patients [76.5 (71.0-80.3) years; 48.3% male] were included in this study. During a median follow-up period of 989 (578-1571) days, 64 patients experienced AS-related events. Patients with low-gradient severe AS had significantly lower event-free survival rates than those with moderate AS (log-rank test, P < 0.001). Multivariable Cox regression analysis showed that the mitral E/e' ratio during exercise was independently associated with AS-related events (hazard ratio = 1.075, P < 0.001) in patients with low-gradient severe AS. Conclusion: This study suggests that asymptomatic patients with low-gradient severe AS have worse prognoses than those with moderate AS. Additionally, the mitral E/e' ratio during exercise is a useful parameter for risk stratification in patients with low-gradient severe AS. [ABSTRACT FROM AUTHOR]

Objectives: Left ventricle function directly impacts left atrial (LA) conduit function, and LA conduit strain is associated with exercise intolerance in patients with heart failure with preserved ejection fraction (HFpEF). Pulmonary capillary wedge pressure (PCWP) before and during exercise is the current gold standard for diagnosing HFpEF. Post-exercise ΔPCWP can lead to worse long-term outcomes. This study examined the correlation between LA strain and post-exercise ΔPCWP in patients with HFpEF. Methods: We enrolled 100 subjects, including 74 with HFpEF and 26 with non-cardiac dyspnea, from November 2017 to December 2020. Subjects underwent echocardiography, invasive cardiac catheterization, and expired gas analysis at rest and during exercise. Arterial blood pressure, right atrial pressure, pulmonary artery pressure, and PCWP were recorded during cardiac catheterization. Cardiac output, stroke volume, pulmonary vascular resistance, pulmonary artery compliance, systemic vascular resistance, and LV stroke work were calculated using standard formulas. Results: Exercise LA conduit strain significantly correlated with both post-exercise ΔPCWP (r = − 0.707, p < 0.001) and exercise PCWP (r = − 0.659; p < 0.001). Exercise LA conduit strain differentiated patients who did and did not meet the 2016 European Society of Cardiology HFpEF criteria with an area under the curve of 0.69 (95% confidence interval, 0.548–0.831) using a cutoff value of 14.25, with a sensitivity of 0.64 and a specificity of 0.68. Conclusions: Exercise LA conduit strain significantly correlates with post-exercise ΔPCWP and has a comparable power to identify patients with HFpEF. Additional studies are warranted to confirm the ability of LA conduit strain to predict long-term outcomes among patients with HFpEF. Clinical relevance statement: Exercise left atrial conduit strain was highly associated with the difference of post-exercise pulmonary capillary wedge pressure and may indicate increased mortality risk in patients with heart failure with preserved ejection fraction, and also has comparable diagnostic ability. Key Points: • Left atrial conduit strain is associated with exercise intolerance in patients with heart failure with preserved ejection fraction. • Left atrial conduit strain during exercise can identify patients with heart failure with preserved ejection fraction. • Exercise left atrial conduit strain significantly correlates with the difference of pulmonary capillary wedge pressure during and before exercise which might predict the long-term outcomes of heart failure with preserved ejection fraction patients. [ABSTRACT FROM AUTHOR]

Left atrial (LA) dilatation is a strong predictor of cardiovascular morbidity and mortality. Strain parameters evaluating LA functions have gained importance in assessing cardiovascular health. This study aimed to investigate the relationship between LA and left ventricular (LV) strain, using speckle-tracking echocardiography, and assess the impact of vena contracta on LV functioning in asymptomatic children with mitral regurgitation. We evaluated 45 asymptomatic mitral regurgitation patients, categorized as mild, moderate, and severe. LV strain was significantly lower in the moderate group (–17.8 ± 3.7%) and further reduced in the severe group (16 ± 2.1%) compared to the reference group (20.5 ± 6.2%). Conversely, LA reservoir strain was higher in the severe and moderate groups (38 ± 12.9% and 40.7 ± 9.5%, respectively) compared to the controls (31.3 ± 7.5%; p < 0.0001). The severe group also displayed a milder depression of contraction (12.2%) compared to the moderate group (13 ± 7.2%). In the moderate and severe groups, LV strain correlated significantly with vena contracta (r=–0.858; p = 0.04), with vena contracta emerging as the only independent predictor for LV longitudinal strain. ROC curve analysis indicated a cut-off value > 5.65 mm for vena contracta, which had a positive predictive value of 75% for predicting LV function impairment. In conclusion, this study emphasizes the significance of assessing LA and ventricular strain in asymptomatic children with mitral regurgitation. Furthermore, vena contracta with diameters greater than 5.6 mm may alert clinicians to the possibility of LV function impairment. These findings offer valuable insights for the follow-up and management of paediatric patients with mitral regurgitation, enabling early detection of potential complications and improved patient outcomes. [ABSTRACT FROM AUTHOR]

Objective: This study aims to investigate the characteristics, risk factors, and outcomes of digital gangrenes in SSc patients, and to identify whether vasculitis is one of the causes for digital gangrene. Methods: A retrospective case–control study was performed from February 2003 to April 2021. Forty-three SSc patients with digital gangrene admitted to Peking Union Medical College Hospital were included. One-hundred forty-six age- and sex-matched SSc patients without gangrene were selected as controls during the same period. Univariate and multivariate logistic regression analysis was used to determine risk factors. Results: Among 43 SSc patients with gangrene, 93.0% had Raynaud's phenomenon (RP) and 32.6% had current or previous digital ulcers (DU). SSc patients with digital gangrene had more ESR elevation (54.8% vs. 34.9%, p = 0.020) and higher level of high-sensitive C reactive protein (median 7.2 mg/L vs. 1.8 mg/L, p = 0.045) compared with controls. In the multivariable logistic regression analysis, smoking history (OR 4.119, p = 0.037), anti-centromere antibody positivity (OR 3.542, p = 0.016), anti-neutrophil cytoplasmic antibody positivity (OR 22.605, p = 0.037), and anti-phospholipid antibody positivity (OR 16.563, p = 0.001), as well as elevated ESR (OR 2.524, p = 0.038) were identified as independent risk factors for gangrenes. Most (79.1%) cases were treated with combination of immunosuppressive and vasodilating therapy, and four cases also got remised after treatment of only glucocorticoid and immunosuppressive agent. Conclusion: Smoking history; positive-ACA, ANCA, and anti-phospholipid antibodies; and increased ESR were independent risk factors for digital gangrenes in SSc. Vasculitis and macrovascular disease may contribute to the progression of digital gangrenes. Key Points •18.6% of SSc patients with digital gangrene had macrovascular stenosis. •Smoking, positive-ACA, ANCA, aPL, and increased ESR were indicators for digital gangrenes in SSc. •Vasculitis and macrovascular disease may involve in the pathogenesis. [ABSTRACT FROM AUTHOR]

Objective: We sought to explore whether classification of patients with heart failure and mid-range (HFmrEF) or preserved ejection fraction (HFpEF) according to their left ventricular ejection fraction (LVEF) identifies differences in their exercise hemodynamic profile, and whether classification according to an index of right ventricular (RV) function improves differentiation., Background: Patients with HFmrEF and HFpEF have hemodynamic compromise on exertion. The classification according to LVEF implies a key role of the left ventricle. However, RV involvement in exercise limitation is increasingly recognized. The tricuspid annular plane systolic excursion/systolic pulmonary arterial pressure (TAPSE/PASP) ratio is an index of RV and pulmonary vascular function. Whether exercise hemodynamics differ more between HFmrEF and HFpEF than between TAPSE/PASP tertiles is unknown., Methods: We analyzed 166 patients with HFpEF (LVEF ≥ 50%) or HFmrEF (LVEF 40-49%) who underwent basic diagnostics (laboratory testing, echocardiography at rest, and cardiopulmonary exercise testing [CPET]) and exercise with right heart catheterization. Hemodynamics were compared according to echocardiographic left ventricular or RV function., Results: Exercise hemodynamics (e.g. pulmonary arterial wedge pressure/cardiac output [CO] slope, CO increase during exercise, and maximum total pulmonary resistance) showed no difference between HFpEF and HFmrEF, but significantly differed across TAPSE/PASP tertiles and were associated with CPET results. N-terminal pro-brain natriuretic peptide concentration also differed significantly across TAPSE/PASP tertiles but not between HFpEF and HFmrEF., Conclusion: In patients with HFpEF or HFmrEF, TAPSE/PASP emerged as a more appropriate stratification parameter than LVEF to predict clinically relevant impairment of exercise hemodynamics. Stratification of exercise hemodynamics in patients with HFpEF or HFmrEF according to LVEF or TAPSE/PASP, showing significant distinctions only with the RV-based strategy. All data are shown as median [upper limit of interquartile range] and were calculated using the independent-samples Mann-Whitney U test or Kruskal-Wallis test. PVR pulmonary vascular resistance; max maximum level during exercise., (© 2021. Springer-Verlag GmbH Germany, part of Springer Nature.)

Aims: The clinical and prognostic importance of functional mitral regurgitation (FMR) in heart failure patients with reduced ejection fraction (HFrEF) has been highly debated. This study aims to define FMR linkage to cardiovascular (CV) outcomes and the interplay with left atrial (LA) function in a prospective cohort of consecutive HFrEF outpatients., Methods and Results: Overall, 286 consecutive outpatients with chronic HFrEF were prospectively enrolled. FMR was quantified by effective regurgitant orifice area (EROA). Global peak atrial longitudinal strain (PALS) was measured by speckle tracking echocardiography. The primary endpoint was a composite of congestive heart failure hospitalization or CV death. During a mean follow-up of 4.1 ± 1.5 years, the primary endpoint occurred in 99 patients (35%). The spline modelling of the risk by FMR severity showed an excess event risk starting at about the EROA value of 0.1 cm 2 . There was a remarkable graded association between the EROA strata, even if tested per 0.1 cm 2 increase, and the risk of CV events (hazard ratio [HR] EROA per 0.10 cm 2 increase: 1.42, 95% confidence interval [CI] 1.19-1.68; p < 0.0001). EROA ≥0.30 cm 2 was associated with CV events regardless of LA function (HR 2.34, 95% CI 1.29-4.19; p = 0.005). Less severe FMR (EROA ≥0.10 cm 2 ) was associated with a dismal outcome only in patients with reduced LA function (PALS <14%) (5-year CV event rate 51 ± 4%); conversely, the risk of events was relative reduced when preserved global PALS and FMR coexisted (5-year CV event rate 38 ± 6%)., Conclusions: Our results refine the independent association between FMR and CV outcome among HFrEF outpatients. Within a moderate EROA range, LA function mitigates the clinical consequences of mitral regurgitation, providing measurable proof of the interplay between regurgitation and LA compliance., (© 2022 European Society of Cardiology.)

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause that leads to respiratory failure and death within few years of diagnosis. Pulmonary hypertension (PH) is a common complication in IPF, where it is strongly associated with increased morbidity and mortality. Patients with IPF and PH have particularly poor prognosis, despite current best medical therapies and the anti-fibrotic therapy with pirfenidone or nintedanib. The aim of our study was to assess the clinical and prognostic impact of PH in patients affected by IPF, already treated with pirfenidone or nintedanib. Seventy-four consecutive outpatients with a diagnosis of IPF, in therapy with pirfenidone or nintedanib, were prospectively enrolled in the study. All patients underwent pulmonary and cardiology assessment by clinical exam, spirometry, DLCO test, chest CT, 6MWT and echocardiography performed by a cardiologist experienced in PH in an ambulatory setting under resting conditions. GAP index has been determinate for all patients. During follow-up, all patients were evaluated every 6 months, or less if necessary. Data about mortality were then collected in a 3-year follow-up. Of the seventy-four patients enrolled, 38 were treated with pirfenidone and 36 with nintedanib. The two groups were comparable for age, gender, FVC, DLCO and PAPS. The patients were also divided in four groups, based on presence of mild/moderate/severe PH by echocardiography at baseline. Significant differences were found for DLCO and the GAP index. Severity of PH was significantly associated with a reduction of DLCO and with an increased GAP index. Survival was directly correlated with 6MWT (R = 0.48), DLCO (R = 0.29, p < 0.01), and reversely with tGAP index (- 0.31, p < 0.01 in all cases), while no significant correlation was found with PAsP. 36-month survival analysis showed an HR of 4.05 (95% CI 1.07-7.34, p = 0.02) for DLCO < 50% and of 1.56 (95% CI 1.02-2.39, p = 0.03) for GAP index. The development and progression of PH in patients affected by IPF reduce the survival and the severity of PH is associated with a reduction of DLCO value and an increase of the GAP index. Echocardiographic stratification based on PAsP values may be useful in stratifying prognosis in IPF patients and deciding specific PAH drugs., (© 2021. Società Italiana di Medicina Interna (SIMI).)

Interstitial lung disease and cardiac involvement are common manifestations and prognostic factors of systemic sclerosis. However, it is unclear whether impaired right atrial function associated with interstitial lung disease in systemic sclerosis can be used as a prognostic factor in this patient population. Therefore, this study aimed to investigate the relationship between right atrial function, interstitial lung disease, and prognosis in patients with systemic sclerosis using tissue tracking analysis with cine cardiac magnetic resonance imaging. In this retrospective observational study, a total of 40 patients with systemic sclerosis were enrolled. Tissue tracking analysis was used to obtain time curves of right atrial strain. Reservoir (total strain), conduit (passive strain), and booster (active strain) pump function were calculated, and right atrial strain, interstitial lung disease, and clinical outcomes were examined. An adverse clinical event was defined as all-cause death. Overall, 23 patients had interstitial lung disease (58%). Six patients died during the follow-up (median, 44 months). The total skin score and right ventricular systolic pressure on echocardiography were higher in patients with an event than in those without an event (28 ± 16% vs. 13 ± 13%, P = 0.02; 46.3 ± 10.7 mmHg vs. 36.0 ± 8.5 mmHg, P = 0.01, respectively). Further, right atrial total strain and active strain were significantly lower in patients with an event than in those without an event (14.3 ± 11.3% vs. 25.8 ± 11.4%, P = 0.03; 3.48 ± 2.37 vs. 11.7 ± 6.78, P = 0.007, respectively). Multivariate analysis revealed that active strain was an independent predictor of all-cause death (hazard ratio 0.76, P = 0.029). Kaplan–Meier analysis revealed that the survival rate was significantly higher in patients with right atrial active strain levels above the cutoff 7.4 (P < 0.05). In systemic sclerosis, right atrial booster function was predictive of mortality. Hence, right atrial functional assessment may have incremental prognostic value for patients with systemic sclerosis, leading to better risk stratification. [ABSTRACT FROM AUTHOR]

Purpose: Mitral valve prolapse (MVP) is associated with left ventricle (LV) fibrosis, including the papillary muscles (PM), which is in turn linked to malignant arrhythmias. This study aims to evaluate comprehensive tissue characterization of the PM by cardiovascular magnetic resonance (CMR) imaging and its association with LV fibrosis observed by intraoperative biopsies. Methods: MVP patients with indication for surgery due to severe mitral regurgitation (n = 19) underwent a preoperative CMR with characterization of the PM: dark-appearance on cine, T1 mapping, conventional bright blood (BB) and dark blood (DB) late gadolinium enhancement (LGE). CMR T1 mapping was performed on 21 healthy volunteers as controls. LV inferobasal myocardial biopsies were obtained in MVP patients and compared to CMR findings. Results: MVP patients (54 ± 10 years old, 14 male) had a dark-appearance of the PM with higher native T1 and extracellular volume (ECV) values compared with healthy volunteers (1096 ± 78ms vs. 994 ± 54ms and 33.9 ± 5.6% vs. 25.9 ± 3.1%, respectively, p < 0.001). Seventeen MVP patients (89.5%) had fibrosis by biopsy. BB-LGE + in LV and PM was identified in 5 (26.3%) patients, while DB-LGE + was observed in LV in 9 (47.4%) and in PM in 15 (78.9%) patients. DB-LGE + in PM was the only technique that showed no difference with detection of LV fibrosis by biopsy. Posteromedial PM was more frequently affected than the anterolateral (73.7% vs. 36.8%, p = 0.039) and correlated with biopsy-proven LV fibrosis (Rho 0.529, p = 0.029). Conclusions: CMR imaging in MVP patients referred for surgery shows a dark-appearance of the PM with higher T1 and ECV values compared with healthy volunteers. The presence of a positive DB-LGE at the posteromedial PM by CMR may serve as a better predictor of biopsy-proven LV inferobasal fibrosis than conventional CMR techniques. [ABSTRACT FROM AUTHOR]