Methamphetamine is increasingly recognized as a cause of pulmonary arterial hypertension (PAH). This study examines whether non-invasively measured metrics of right heart function, right atrial (RA) and right ventricular (RV) strain, are more impaired in methamphetamine-associated PAH (MA-PAH) compared with idiopathic PAH (IPAH).

A retrospective cohort analysis of 51 patients with MA-PAH matched for mean pulmonary artery pressure (mPAP) with 51 patients with IPAH followed at the pulmonary hypertension clinic at the University of Utah was performed. Invasive hemodynamics and echocardiographic measures of right heart function, including RA strain and RV free wall strain, were compared.

Compared to the matched IPAH group, MA-PAH patients had lower cardiac index (2.04 ± 0.84 vs. 2.52 ± 1.07 L/min/m2, p = 0.016) and higher pulmonary vascular resistance (PVR; 11.8 ± 6.8 vs. 8.9 ± 4.8 Wood units, p = 0.018). The MA-PAH group had larger RA maximal and minimal volume, lower RA reservoir strain (26.4 ± 11.7 vs. 33.4 ± 14.8 %, p = 0.011), more significant RV chamber dilation, and lower fractional area change (FAC; 21.1 ± 11.1 % vs. 34.5 ± 11.8 %, p < 0.001), compared to the IPAH group. RV e' was lower in MA-PAH (6.5 ± 2.8 cm/s vs. 8.3 ± 4.3 cm/s, p = 0.021), suggesting worse RV diastolic function and RV free wall strain was significantly more reduced compared to patients with I-PAH (17.0 ± 6.5 vs. 22.3 ± 7.2 %, p < 0.001). There were no differences in 5-year survival (p = 0.26), 6MW distance including stratification for males and females (p = 0.249 in females, p = 0.279 in males), and rehospitalization rates within 5 years of diagnosis (p = 0.70).

Despite a similar mPAP, patients with MA-PAH had more RA dilation, RV dilation, lower RV systolic/diastolic function, and worse RA and RV mechanics as assessed by strain compared to patients with I-PAH. Our findings suggest that, in addition to causing remodeling of the pulmonary vasculature, methamphetamine may have a direct cardiotoxic effect on the right heart.

Sleep-disordered breathing (SDB), comprising obstructive sleep apnea (OSA) and central sleep apnea (CSA), could promote left ventricular (LV) remodeling and systolic dysfunction.

The authors tested the hypothesis, in the ADVENT-HF (Adaptive Servo-ventilation for Sleep-disordered Breathing in Patients with Heart Failure with Reduced Ejection Fraction) trial, that SDB is associated with reversible impairment of LV structure and function.

Participants underwent echocardiography (ejection fraction ≤45%) and polysomnography. They were classified as no-SDB (apnea-hypopnea index [AHI] <15/h); OSA (AHI ≥15/h with ≥50% of events obstructive), or CSA (AHI ≥15 with >50% of events central). Those with SDB were randomized to a control group or to peak-flow-triggered adaptive servo-ventilation (ASVPF) to treat SDB and, in them, echocardiography was repeated 6 months later.

Subjects with OSA (n = 543) had similar LV structure and function to those without SDB (n = 56). Subjects with CSA (n = 201) had greater LV mass index and lower LV ejection fraction than the other groups. LV volumes were higher in the CSA than in the OSA group (P < 0.05 for all). ASVPF abolished SDB, but had no significant effect, whether evaluated by allocation or adherence, 6 months post-randomization on LV structure or function for the entire cohort (n = 549), or either subgroup.

Among patients with heart failure with reduced ejection fraction, those with OSA had similar LV structure and function to those without SDB. Patients with CSA had greater LV remodeling and systolic dysfunction than those with OSA or without SDB. Six months of SDB suppression of OSA and CSA by ASVPF had no impact on LV structure or function. (Adaptive Servo-ventilation for Sleep-disordered Breathing in Patients with Heart Failure with Reduced Ejection Fraction [ADVENT-HF]).

Despite a near universal approach focused on physiologic stabilization prior to surgical repair of congenital diaphragmatic hernia (CDH), a condition with significant morbidity and mortality, there remains a lack of consensus, or even guidance, on the appropriate timing for diaphragmatic reconstruction. Hemodynamic-directed care has increasingly been incorporated into the post-natal, peri-operative care of CDH, including assessments of pulmonary hypertension and ventricular dysfunction. Herein, we discuss the integration of targeted neonatal echocardiography and hemodynamic-based assessments to guide precision-directed care and inform selection of the optimal surgical repair window in this complex, heterogeneous patient population.

Delayed diagnosis of pulmonary hypertension (PH) is a known cause of poor patient prognosis. We aimed to develop an artificial intelligence (AI) model, using ensemble learning method to detect PH using electrocardiography (ECG), chest X-ray (CXR), and brain natriuretic peptide (BNP), facilitating accurate detection and prompting further examinations.

We developed a convolutional neural network model using ECG data to predict PH, labelled by ECG from seven institutions. Logistic regression was used for the BNP prediction model. We referenced a CXR deep learning model using ResNet18. Outputs from each of the three models were integrated into a three-layer fully connected multimodal model. Ten cardiologists participated in an interpretation test, detecting PH from patients' ECG, CXR, and BNP data both with and without the ensemble learning model. The area under the receiver operating characteristic curves of the ECG, CXR, BNP, and ensemble learning model were 0.818 [95% confidence interval (CI), 0.808-0.828], 0.823 (95% CI, 0.780-0.866), 0.724 (95% CI, 0.668-0.780), and 0.872 (95% CI, 0.829-0.915). Cardiologists' average accuracy rates were 65.0 ± 4.7% for test without AI model and 74.0 ± 2.7% for test with AI model, a statistically significant improvement (P < 0.01).

Our ensemble learning model improved doctors' accuracy in detecting PH from ECG, CXR, and BNP examinations. This suggests that earlier and more accurate PH diagnosis is possible, potentially improving patient prognosis.

Background/Objectives: Pulmonary arterial hypertension (PAH) is a progressive vascular disorder characterized by increased pulmonary vascular resistance, right ventricular dysfunction, and high mortality rates. Despite advancements in vasodilatory therapies, PAH remains a life-threatening condition with limited curative options. This review aimed to explore emerging molecular mechanisms, novel therapeutic targets, and future research directions in PAH treatment, focusing on strategies to improve long-term patient outcomes. Methods: This review synthesized recent advancements in PAH pathophysiology and therapeutic development. A structured literature search was conducted on PubMed and ClinicalTrials.gov using keywords such as "Pulmonary Arterial Hypertension", "vascular remodeling", "metabolic dysfunction", and "emerging therapies". Studies published between 2015 and 2025 were included, with a focus on preclinical models, clinical trials, and translational research. Key areas of investigation include vascular remodeling, metabolic dysregulation, inflammation, and right ventricular dysfunction. The review also evaluated the potential of novel pharmacological agents, gene-based therapies, and AI-driven diagnostics for PAH management. Results: Recent studies highlight dysregulated BMPR2 signaling, epigenetic modifications, and inflammatory cytokine pathways as critical contributors to PAH progression. Emerging therapies such as JAK-STAT inhibitors, metabolic reprogramming agents, and mesenchymal stromal cell-derived extracellular vesicles (EVs) show promise in preclinical and early clinical trials. Additionally, AI-enhanced imaging and non-invasive biomarkers are improving PAH diagnostics. Future research directions emphasize precision medicine approaches and the development of RV-targeted therapies. Conclusions: PAH remains a complex and fatal disease requiring multifaceted therapeutic strategies beyond traditional vasodilation. Advances in molecular-targeted treatments, AI-driven diagnostics, and personalized medicine offer new hope for disease-modifying interventions. Future research must bridge translational gaps to bring novel therapies from bench to bedside, improving survival and quality of life in PAH patients.

To investigate alterations in blood lipid profiles and T cell subsets among systemic sclerosis (SSc) patients, and to assess their potential utility in predicting cardiovascular disease (CVD) risk.

105 SSc patients and 80 age- and sex-matched healthy controls (HCs) were enrolled. Flow cytometry was employed to quantify T cell subsets. Multivariate logistic regression analysis investigated the association between blood lipid profile, T cell subsets, SSc occurrence, and CVD risk. Additionally, a prediction model was developed to assess the potential predictive value of CVD risk.

In the SSc patients, low-density lipoprotein cholesterol (LDL-C) (OR = 3.212, 95%CI = 1.132-9.113, p= 0.028), ESR (OR = 1.218, 95%CI = 1.086-1.367, p= 0.001), CRP (OR = 2.156, 95% CI = 1.393-3.338, p = 0.001), T helper (Th)cells (OR = 1.004, 95% CI = 1.001-1.008, p = 0.034) were positively correlated with the risk of SSc. Further studies found that absolute increases in Th cells in SSc patients were positively associated with the risk of CVD (OR=1.002, 95%CI=1.001-1.005, p =0.011) and were independent predictors of CVD risk in SSc. When Th cells exceeded 866.53 cells/μL, the risk of CVD in SSc patients was greatly increased (p<0.001).

Altered lipid profiles and dysregulated Th cell expression in SSc patients, with a significant elevation of Th cells specifically noted in SSc-CVD patients, suggesting that Th cells may serve as a potential predictive biomarker for CVD in SSc patients, thereby aiding in early diagnosis. The underlying mechanism of this association requires further investigation.

Sarcoidosis is a granulomatous disorder of unknown etiology characterized by multisystem non-caseating granulomas. Pulmonary hypertension (PH) is a well-known complication of sarcoidosis and is associated with increased morbidity and mortality. The actual epidemiology of sarcoidosis-associated PH (SAPH) remains unknown, and its pathogenesis has not been fully elucidated. SAPH is classified under the miscellaneous category (group 5 of the PH classification). The clinical presentation of SAPH is variable and not always proportional to the severity of sarcoidosis. Appropriate management for SAPH by an experienced physician is important; however, no treatment algorithm for SAPH has been established. Lung transplantation should be considered in refractory cases. Pulmonary arterial hypertension-specific vasodilators targeting the endothelin pathway, nitric oxide pathway, and prostacyclin pathway have improved the clinical functions and hemodynamics in some patients with SAPH.

The aim of this study was to investigate lymphocyte subsets, especially natural killer (NK) cells, in patients with systemic sclerosis (SSc) and evaluate the diagnostic value of NK cells in secondary pulmonary arterial hypertension (PAH). A total of 115 SSc patients and 100 age- and sex-matched health controls (HCs) were enrolled in this study. Flow cytometry was employed to quantify NK cells, while the association between NK cells and disease activity as well as PAH was investigated to further elucidate its diagnostic potential. The absolute count of NK (CD3-CD56+) cells significantly decreased in SSc patients. There was a negative correlation between the mRSS score and the injury index. The levels of cytokine exhibited significant elevation among SSc patients. Conversely, SSc-PAH patients demonstrated significantly elevated levels of CRP, UA, and BNP. Additionally, there was a significant reduction in the absolute level of NK cells. ROC curve analysis revealed that the optimal cut-off point for NK cells was 185 cells/µL, while for BNP it was 70.50 pg/mL and for UA it was 323.00 µmol/L. Our study revealed a significant inverse correlation between peripheral blood NK cell levels and the incidence of complicated PAH in patients with SSc.

In patients with significant tricuspid regurgitation, cardiac magnetic resonance imaging (CMR) is the preferred method for the evaluation of right ventricular function and volumes. However validated thresholds are lacking.

The aim of this study was to evaluate CMR assessment of right ventricular volumes in patients with significant (moderate or severe) tricuspid regurgitation, and to define its association with outcomes.

The PRONOVAL study is a retrospective multicentre study using the clinical data warehouse of Greater Paris University Hospitals (AP-HP). Patients were screened for CMR in the PMSI (Programme de médicalisation des systèmes d'information). Hospitalization reports were analysed by natural language processing to include patients with tricuspid regurgitation. Exclusion criteria were left heart valvular disease, heart transplantation and cardiac amyloidosis. Primary outcome was a combined criterion of death or tricuspid surgery.

Between September 2017 and September 2021, 151 patients with isolated tricuspid regurgitation were screened. Right ventricular function and volumes were available in 86 (57.0%) CMR reports (the complete CMR group). In the complete CMR group, tricuspid regurgitation was severe in 62 patients (72.1%). Median age was 67.0 years (interquartile range 58.0-75.8). Median right ventricular indexed end-diastolic volume was 98.0 mL/m2 (interquartile range 66.8-118.5). At 2-year follow-up, six patients (9.2%) had undergone tricuspid valve surgery, and 12 patients (18.5%) had died. Right ventricular indexed end-diastolic volume was associated with death or surgery at 2years, with an area under the receiver operating characteristic curve of 0.76 (95% confidence interval 0.75-0.77) for a threshold of 119mL/m2.

Right ventricular indexed end-diastolic volume >119mL/m2 was found to be an independent indicator of death or surgery in patients with significant tricuspid regurgitation.

Transcatheter aortic valve replacement (TAVR) has become a viable alternative to surgical aortic valve replacement (SAVR) for high-risk patients with aortic stenosis. One such high-risk group is patients with pulmonary hypertension (PH), which is known to increase surgical risk and adversely affect outcomes. This study aims to compare midterm and long-term survival in TAVR and SAVR among patients with PH.

A retrospective review of patients with PH undergoing an aortic valve replacement was conducted at a single institution. From May 2012 to June 2020, 427 patients with PH underwent a primary isolated TAVR (n = 249) or SAVR (n = 178). PH was assessed using an estimated pulmonary artery systolic pressure ≥40 mmHg. Propensity score matching was used to adjust for confounders.

Before matching, Kaplan-Meier estimated survival was significantly lower for TAVR compared to SAVR (P < 0.01). After matching, 87 well-balanced pairs remained. Post propensity score matching, Kaplan-Meier estimated survival was still significantly lower for the TAVR group as compared to the SAVR group (P = 0.045).

These data support the feasibility and safety of SAVR in selected patients with PH, and the presence of PH should not preclude consideration of SAVR in appropriate surgical candidates. Further multicenter research is needed to explore confounders and deepen our understanding of the long-term outcomes in this high-risk population.

Pediatric pulmonary hypertension (PH) is characterized by a mean pulmonary arterial pressure exceeding 20 mmHg. There is limited research on the suitability of adult-based methods for estimating PH in pediatric populations. Using established formulas for adults, this study aimed to evaluate the correlation between echocardiographic estimates of systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures in children with congenital heart disease (CHD).

A prospective study was conducted involving children with CHD undergoing cardiac catheterization without prior cardiac surgery. We used echocardiography to estimate pulmonary and right atrial pressures and compared these with invasively measured values. Four reliable regression equations were developed to estimate systolic, diastolic, and mean pulmonary arterial pressures, and mean right atrial pressures. Cutoff values were determined to predict the occurrence of PH. Linear regression, Bland-Altman analysis, and receiver operating characteristic curve analysis were performed to assess the accuracy of echocardiography and establish diagnostic thresholds for PH.

The study involved 55 children (23 with normal pulmonary arterial pressure and 32 with PH) with acyanotic CHD aged 1 to 192 months. Four equations were developed to detect high pulmonary arterial pressures, with cutoff values of 32.9 for systolic pulmonary arterial pressure, 14.95 for diastolic pulmonary arterial pressure, and 20.7 for mean pulmonary arterial pressure. The results showed high sensitivity and moderate specificity but a tendency to underestimate systolic and mean pulmonary arterial pressures at higher pressures.

The study provides valuable insights into the use of adult-based echocardiographic formulas for estimating PH in pediatric patients with acyanotic CHD.

The tricuspid regurgitation pressure gradient (TRPG) reflects the difference in pressure between the right ventricle and right atrium (ΔPRV-RA). Its estimation by echocardiography correlates well with that obtained using right-heart catheterization. An elevated TRPG is an important marker for identifying right ventricular dysfunction in both the acute and chronic settings. However, in the "wide-open" variant of TR, the TRPG counterintuitively falls. Failure to recognize this potential pitfall and underlying pathophysiology can cause underestimation of the severity of right ventricular dysfunction. This could lead to erroneous fluid tolerance assessments, and potentially harmful resuscitative and airway management strategies. In this manuscript, we illustrate the pathophysiology and potential pitfall of wide-open TR through a series of cases in which emergency physicians made the diagnosis using cardiac point-of-care ultrasound. To our knowledge, this clinical series is the first to demonstrate recognition of the paradoxically-low TRPG of wide-open TR, which guided appropriate management of critically ill patients in the emergency department.

The association of pulmonary hypertension (PH) with the outcome after mitral transcatheter edge-to-edge repair (M-TEER) focusing on the new ESC/ERS guidelines definition for PH.

PH is frequently found in patients with mitral regurgitation and is associated with lower survival rates. Recent studies were based on echocardiographic parameters, but results based on invasive haemodynamics differentiating distinct types of PH using the new definition for PH are missing.

449 consecutive M-TEER-treated patients from December 2009 to February 2015 were included in this retrospective analysis. All patients were stratified by the distinct types of PH (no PH, precapillary PH, isolated postcapillary PH, combined post-PH and precapillary PH) according to the definitions of the ESC/ERS guidelines for the diagnosis of PH from 2015 (meanPA cut-off <25 mm Hg, pulmonary capillary wedge pressure (PCWP) cut-off ≤15 mm Hg, diastolic pulmonary gradient cut-off ≥7 mm Hg or pulmonary vascular resistance (PVR) >3 WU) and 2022 (meanPA cut-off ≤20 mm Hg, PCWP cut-off ≤15 mm Hg, PVR cut-off ≥3 WU).

Patients with any type of PH (2015: meanPA cut-off 25 mm Hg; 2022: meanPA cut-off >20 mm Hg) showed a higher risk of death after M-TEER compared with patients with no PH (2015: HR 1.61 (95% CI 1.25 to 2.07); p<0.001 and 2022: HR 2.09 (95% CI 1.54 to 2.83); p<0.001). Based on the new PH definition, each PH subgroup showed a lower survival after M-TEER compared with patients with no PH. Echocardiographic estimated systolic PAP showed a correlation with invasively measured mean pulmonary artery pressure (mPAP) (r=0.29, p<0.001) and systolic pulmonary arterial pressure (r=0.34,p<0.001). Cox-regression analysis showed higher invasive diastolic, systolic and mean pulmonary pressures were associated with higher all-cause mortality (p<0.001). In addition, invasive measured higher right atrial pressure, lower pulmonary arterial compliance, higher PVR and higher wedge pressure were identified as predictors of all-cause mortality after M-TEER.

The new PH definition discriminates PH groups and mortality better than the old definition. The lower threshold of mPAP of 20mmHg improved prognostication in this cohort of patients.

Pulmonary arterial hypertension (PAH) is a chronic and progressive disease that eventually leads to heart failure (HF) and subsequent fatality if left untreated. Right ventricular (RV) function has proven prognostic values in patients with a variety of heart diseases including PAH. PAH is predominantly a right heart disease; however, given the nature of the continuous circulatory system and the presence of shared septum and pericardial constraints, the interdependence of the right and left ventricles is a factor that requires consideration. Accurate and timely assessment of ventricular function is very important in the management of patients with PAH for disease outcomes and prognosis. Non-invasive modalities such as cardiac magnetic resonance (CMR) and echocardiography (two-dimensional and three-dimensional), and nuclear medicine, positron emission tomography (PET) play a crucial role in the assessment of ventricular function and disease prognosis. Each modality has its own strengths and limitations, hence this review article sheds light on (i) ventricular dysfunction in patients with PAH and RV-LV interdependence in such patients, (ii) the strengths and limitations of all available modalities and parameters for the early assessment of ventricular function, as well as their prognostic value, and (iii) lastly, the challenges faced and the potential future advancement in these modalities for accurate and early diagnosis of ventricular function in PAH.

The right ventricular (RV) function correlates with prognosis in severe pulmonary artery hypertension (PAH) but which metric of it is most clinically relevant is still uncertain. Clinical methods to estimate RV function from simplified pressure volume loops correlate with disease severity but the clinical relevance has not been assessed. Evaluation of right ventricle pulmonary artery coupling in pulmonary hypertensive patients may help to elucidate the mechanisms of right ventricular failure and may also help to identify patients at risk or guide the timing of therapeutic interventions in pulmonary hypertension. Complete evaluation of RV failure requires echocardiographic or magnetic resonance imaging, and right heart catheterization measurements. Treatment of RV failure in PAH relies on decreasing afterload with drugs targeting pulmonary circulation; fluid management to optimize ventricular diastolic interactions; and inotropic interventions to reverse cardiogenic shock. The ability to relate quantitative metrics of RV function in pulmonary artery hypertension to clinical outcomes can provide a powerful tool for management. Such metrics could also be utilized in the future as surrogate endpoints for outcomes and evaluation of response to therapies. This review of literature gives an insight on RV-PA coupling associated with PAH, its types of measurement and pharmacological treatment.

Stress echocardiography has been one of the most promising methods for the diagnosis of ischemic heart disease, hypertrophic cardiomyopathy, and pulmonary hypertension. The Japanese Society of Echocardiography produced practical guidance for the implementation of stress echocardiography in 2018. At that time, stress echocardiography was not yet widely disseminated in Japan; therefore, the 2018 practical guidance for the implementation of stress echocardiography included a report on stress echocardiography and a specific protocol to promote its use at many institutions in Japan in the future. And now, an era of renewed interest and enthusiasm surrounding the diagnosis and treatment of valvular heart disease and heart failure with preserved ejection fraction (HFpEF) has come, which are driven by emerging trans-catheter procedures and new recommended guideline-directed medical therapy. Based on the continued evidence of stress echocardiography, the new practical guideline that describes the safe and effective methodology of stress echocardiography is now created by the Guideline Development Committee of the Japanese Society of Echocardiography and is designed to expand the use of stress echocardiography for valvular heart disease and HFpEF, as well as ischemic heart disease, hypertrophic cardiomyopathy, and pulmonary hypertension. The readers are encouraged to perform stress echocardiography which will enhance the diagnosis and management of these patients.

Pulmonary hypertension (PH) is a complex multifactorial vascular pathology characterized by an increased pulmonary arterial pressure, vasoconstriction, remodelling of the pulmonary vasculature, thrombosis in situ and inflammation associated with right-side heart failure. Herein, we explored the potential beneficial effects of treatment with siRNA AP-1 on pulmonary arterial hypertension (PAH), right ventricular dysfunction along with perivascular and interstitial fibrosis in pulmonary artery-PA, right ventricle-RV and lung in an experimental animal model of monocrotaline (MCT)-induced PAH.

Golden Syrian hamsters were divided into: (1) C group-healthy animals taken as control; (2) MCT group obtained by a single subcutaneous injection of 60 mg/kg MCT at the beginning of the experiment; (3) MCT-siRNA AP-1 group received a one-time subcutaneous dose of MCT and subcutaneous injections containing 100 nM siRNA AP-1, every two weeks. All animal groups received water and standard chow ad libitum for 12 weeks.

In comparison with the MCT group, siRNA AP-1 treatment had significant beneficial effects on investigated tissues contributing to: (1) a reduction in TGF-β1/ET-1/IL-1β/TNF-α plasma concentrations; (2) a reduced level of cytosolic ROS production in PA, RV and lung and notable improvements regarding the ultrastructure of these tissues; a decrease of inflammatory and fibrotic marker expressions in PA (COL1A/Fibronectin/Vimentin/α-SMA/CTGF/Calponin/MMP-9), RV and lung (COL1A/CTGF/Fibronectin/α-SMA/F-actin/OB-cadherin) and an increase of endothelial marker expressions (CD31/VE-cadherin) in PA; (4) structural and functional recoveries of the PA [reduced Vel, restored vascular reactivity (NA contraction, ACh relaxation)] and RV (enlarged internal cavity diameter in diastole, increased TAPSE and PRVOFs) associated with a decrease in systolic and diastolic blood pressure, and heart rate; (5) a reduced protein expression profile of AP-1S3/ pFAK/FAK/pERK/ERK and a significant decrease in the expression levels of miRNA-145, miRNA-210, miRNA-21, and miRNA-214 along with an increase of miRNA-124 and miRNA-204.

The siRNA AP-1-based therapy led to an improvement of pulmonary arterial and right ventricular function accompanied by a regression of perivascular and interstitial fibrosis in PA, RV and lung and a down-regulation of key inflammatory and fibrotic markers in MCT-treated hamsters.

(1) Background: The aim was to evaluate the effectiveness of the probiotic containing Saccharomyces boulardii in the treatment of small intestinal bacterial overgrowth (SIBO) in patients with decompensated cirrhosis. (2) Methods: This was a blinded, randomized, placebo-controlled study. (3) Results: After 3 months of treatment, SIBO was absent in 80.0% of patients in the probiotic group and in 23.1% of patients in the placebo group (p = 0.002). The patients with eliminated SIBO had decreased frequency of ascites and hepatic encephalopathy, the increased platelets and albumin levels, the decreased blood levels of total bilirubin, biomarkers of bacterial translocation (lipopolysaccharide [LPS]) and systemic inflammation (C-reactive protein), and positive changes in markers of hyperdynamic circulation compared with the state at inclusion. There were no significant changes in the claudin 3 level (the intestinal barrier biomarker) in these patients. No significant changes were observed in the group of patients with persistent SIBO. The serum level of nitrate (endothelial dysfunction biomarker) was lower in patients with eradicated SIBO than in patients with persistent SIBO. One (5.3%) patient with eradicated SIBO and six (42.9%) patients with persistent SIBO died within the first year of follow-up (p = 0.007). (4) Conclusions: SIBO eradication was an independent predictor of a favorable prognosis during the first year of follow-up.

To evaluate a machine learning (ML)-based model for pulmonary hypertension (PH) prediction using measurements and impressions made during echocardiography.

A total of 7853 consecutive patients with right-sided heart catheterization and transthoracic echocardiography performed within 1 week from January 1, 2012, through December 31, 2019, were included. The data were split into training (n=5024 [64%]), validation (n=1275 [16%]), and testing (n=1554 [20%]). A gradient boosting machine with enumerated grid search for optimization was selected to allow missing data in the boosted trees without imputation. The training target was PH, defined by right-sided heart catheterization as mean pulmonary artery pressure above 20 mm Hg; model performance was maximized relative to area under the receiver operating characteristic curve using 5-fold cross-validation.

Cohort age was 64±14 years; 3467 (44%) were female, and 81% (6323/7853) had PH. The final trained model included 19 characteristics, measurements, or impressions derived from the echocardiogram. In the testing data, the model had high discrimination for the detection of PH (area under the receiver operating characteristic curve, 0.83; 95% CI, 0.80 to 0.85). The model's accuracy, sensitivity, positive predictive value, and negative predictive value were 82% (1267/1554), 88% (1098/1242), 89% (1098/1241), and 54% (169/313), respectively.

By use of ML, PH could be predicted on the basis of clinical and echocardiographic variables, without tricuspid regurgitation velocity. Machine learning methods appear promising for identifying patients with low likelihood of PH.

Apical hypertrophic cardiomyopathy (ApHCM) is a subtype of hypertrophic cardiomyopathy (HCM) that affects up to 25% of Asian patients and is not as well understood in non-Asian patients. Although ApHCM has been considered a more "benign" variant, it is associated with increased risk of atrial and ventricular arrhythmias, apical thrombi, stroke, and progressive heart failure. The occurrence of pulmonary hypertension (PH) in ApHCM, due to elevated pressures on the left side of the heart, has been documented. However, the exact prevalence of PH in ApHCM and sex differences remain uncertain.

We sought to evaluate the prevalence, risk associations, and sex differences in elevated pulmonary pressures in the largest cohort of patients with ApHCM at a single tertiary center. A total of 542 patients diagnosed with ApHCM were identified using ICD codes and clinical notes searches, confirmed by cross-referencing with cardiac MRI reports extracted through Natural Language Processing and through manual evaluation of patient charts and imaging records.

In 414 patients, echocardiogram measurements of pulmonary artery systolic pressure (PASP) were obtained at the time of diagnosis. The mean age was 59.4 ± 16.6 years, with 181 (44%) being females. The mean PASP was 38 ± 12 mmHg in females vs. 33 ± 9 mmHg in males (p < 0.0001). PH as defined by a PASP value of > 36 mmHg was present in 140/414 (34%) patients, with a predominance in females [79/181 (44%)] vs. males [61/233 (26%), p < 0.0001]. Female sex, atrial fibrillation, diagnosis of congestive heart failure, and elevated filling pressures on echocardiogram remained significantly associated with PH (PASP > 36 mmHg) in multivariable modeling. PH, when present, was independently associated with mortality [hazard ratio 1.63, 95% CI (1.05-2.53), p = 0.028] and symptoms [odds ratio 2.28 (1.40, 3.71), p < 0.001].

PH was present in 34% of patients with ApHCM at diagnosis, with female sex predominance. PH in ApHCM was associated with symptoms and increased mortality.

Despite advanced aortic valve replacement techniques, aortic stenosis (AS)-induced irreversible myocardial fibrosis contributes to poorer outcomes. Therefore, in addition to early diagnosis of AS, detecting myocardial fibrosis is crucial for physicians to determine the timing of surgery. The Signal Intensity Coefficient (SIC) was used to detect subtle myocardial deformation. Hence, we aimed to investigate whether SIC correlated with myocardial dysfunction and fibrosis from both clinical and preclinical perspectives.

We collected medical records and echocardiography images, including the SIC of patients who underwent surgical aortic valve replacement (AVR) for AS from 2010 to 2015. The endpoint of the study was mortality. Median follow-up period was 80 months.

Among 109 patients, 15 died due to cardiovascular causes. Although SIC decreased in all patients post-AVR, patients with an SIC ≥0.34 before surgeries presented with a higher probability of cardiovascular death. In contrast, changes in the left ventricular (LV) ejection fraction, LV mass index, and LV volume failed to predict outcomes. Similarly, SIC was obtained in mice undergoing aortic banding and debanding surgery for comparison with the degree of myocardial fibrosis. SIC was continuously elevated after aortic banding and declined gradually after debanding surgery in mice. Debanding surgery indicated the regression of aortic banding-induced myocardial fibrosis.

Pre-AVR SIC was associated with the risk of cardiovascular death and reflected the degree of myocardial fibrosis. Further investigations are required to study the clinical application of SIC in patients with AS.

Pulmonary hypertension is frequent in infants with bronchopulmonary dysplasia. Echocardiography is easy to perform, non-invasive, and recommended by guidelines even though solely it is not enough. Catheterisation is gold standard but invasive, expensive, and not cost effective. Therefore, we aimed to assess to find out the role of biomarkers besides echocardiography in the diagnosis of pulmonary hypertension in preterm with bronchopulmonary dysplasia.

This study is done during the time period January 2016-2017. The diagnosis of pulmonary hypertension was assessed by echocardiography at 36 weeks later repeated at 3rd and 6th months. We also repeated biomarkers at 3rd and 6th months. The infants born ≤ 28 weeks in Erciyes University hospital who were diagnosed bronchopulmonary dysplasia were included. Infants with genetic syndromes, structural lung, and CHDs were excluded. Patients without bronchopulmonary dysplasia but having pulmonary hypertension due to other reasons and patients having echocardiograms without adequate images were excluded.

At initial, 21/59 patients had bronchopulmonary dysplasia-pulmonary hypertension (Group 1), 21/59 had no bronchopulmonary dysplasia-pulmonary hypertension (Group 2), and 17/59 had bronchopulmonary dysplasia without pulmonary hypertension (Group 3). Systolic pulmonary artery pressure and pulmonary vascular resistance were found high in Group 1 (36 mmHg; p <0.001, 1.25 Woods Unit; p < 0.0017, respectively). Tricuspid annular plane systolic excursion values of Group 1 were low. Median serum kallistatin levels of Group 1 were lower than the other groups (230.5 (114.5-300.5) µg/ml; p < 0.005). During the study period, pulmonary hypertension of 14/21 bronchopulmonary dysplasia-pulmonary hypertension resolved, six patients in Group 3 developed pulmonary hypertension. However, there was no difference in the biomarkers of these six patients.

In the diagnosis and the follow-up of pulmonary hypertension in bronchopulmonary dysplasia patients, besides echocardiography kallistatin, gelsolin, NT-probrain natriuretic peptide, homocysteine, and cystatin-C levels can be used. Further studies were required with large sample sizes.

Pulmonary artery hypertension (PAH) is a fatal disease characterized by a complex pathogenesis. Exosomes containing microRNAs (miRs) have emerged as a novel biomarker. Transpulmonary exosomal miRs offer valuable insights into pulmonary circulation microenvironments. Hereby, we aimed to explore the potentials of transpulmonary exosomal miRs as differentiating factors between idiopathic PAH and congenital heart disease (CHD)-related PAH.

During right heart catheterization, we collected exosomes at pulmonary arteries in 25 patients diagnosed with idiopathic PAH and 20 patients with CHD-related PAH. Next-generation sequencing identified several candidate exosomal miRs. Using quantitative polymerase chain reaction, we validated the expressions of these miRs and revealed significantly elevated expressions of miR-21, miR-139-5p, miR-155-5p, let-7f-5p, miR-328-3p, miR-330-3p, and miR-103a-3p in patients with CHD-related PAH, in contrast to patients with idiopathic PAH. Among these miRs, miR-21 exhibited the highest expression in patients with CHD-related PAH. These findings were further corroborated in an external cohort comprising 10 patients with idiopathic PAH and 8 patients with CHD-related PAH. Using an in vitro flow model simulating the shear stress experienced by pulmonary endothelial cells, we observed a significant upregulation of miR-21. Suppressing miR-21 rescued the shear stress-induced downregulation of the RAS/phosphatidylinositol 3-kinase/protein kinase B pathway, leading to a mitigation of apoptosis.

Our study identified a pronounced expression of transpulmonary exosomal miR-21, particularly in patients with CHD-related PAH, through next-generation sequencing analysis. Further investigation is warranted to elucidate the regulatory mechanisms involving miR-21 in the pathophysiology of PAH.

Left heart disease is the most common cause of pulmonary hypertension (PH) and is frequently accompanied by increases in pulmonary vascular resistance. However, the distinction between phenotypes of PH due to left heart disease with a normal or elevated pulmonary vascular resistance-isolated postcapillary PH (IpcPH) and combined pre- and postcapillary PH (CpcPH), respectively-has been incompletely defined using unbiased methods.

Patients with extremes of IpcPH versus CpcPH were identified from a single-center record of those who underwent right heart catheterization. Individuals with left ventricular ejection fraction <40% or with potential causes of PH beyond left heart disease were excluded. Medication usage in IpcPH and CpcPH was compared across Anatomical Therapeutic Chemical classes and identified vitamin K antagonists as the only medication with pharmacome-wide significance, being more commonly used in CpcPH and for an indication of atrial fibrillation in ≈90% of instances. Accordingly, atrial fibrillation prevalence was significantly higher in CpcPH in a phenome-wide analysis. Review of echocardiographic data most proximal to right heart catheterization revealed that left atrial diameter indexed to body surface area-known to be associated with atrial fibrillation-was increased in CpcPH regardless of the presence of atrial fibrillation. An independent cohort with serial right heart catheterizations and PH-left heart disease showed a significant positive correlation between change in left atrial diameter indexed to body surface area and change in pulmonary vascular resistance.

Guided by pharmacomic and phenomic screens in a rigorously phenotyped cohort, we identify a longitudinal association between left atrial diameter indexed to body surface area and pulmonary vascular resistance with implications for the future development of diagnostic, prognostic, and therapeutic tools.

Prognostic implications of pulmonary hypertension (PH) in low-flow low-gradient (LG) aortic stenosis (AS) after transcatheter aortic valve replacement (TAVR) remains unexplored. We aimed to investigate the impact of baseline and changes in PH after TAVR. In this single-center retrospective study, we included patients who underwent TAVR for low-flow LG AS. Patients were categorized into 2 groups: baseline pulmonary artery systolic pressure (PASP) <46 mm Hg (no-to-mild PH) and PASP ≥46 mm Hg (moderate-to-severe PH). On the basis of changes in PASP after TAVR, patients were stratified into increased (ΔPASP ≥ + 5 mm Hg), no change (-4 to +4 mm Hg), and decreased (≤ -5 mm Hg) groups. Primary end point was a composite of all-cause mortality and heart failure rehospitalization. In total, 210 patients were included, 148 in the no-to-mild PH group and 62 in the moderate-to-severe PH group. Median follow-up was 13.2 months. The moderate-to-severe PH group was at an increased risk of composite end point (adjusted hazard ratio [HR] 3.5, 95% confidence interval [CI] 1.8 to 6.9), all-cause mortality (HR 2.4, 95% CI 1.1 to 5.6), and heart failure rehospitalization (HR 8.3, 95% CI 2.9 to 23.7). There were no differences in clinical outcomes among those with increased (32%), no change (28%), and decreased (39%) PASP after TAVR. In conclusion, moderate-to-severe PH at baseline is an independent predictor of worse clinical outcomes in patients with low-flow LG AS who undergo TAVR, and this cohort of patients do not seem to derive the benefits of postoperative reduction of PASP.

To discuss physiologic and methodologic advances in the echocardiographic assessment of right heart (RH) function, including the emergence of artificial intelligence (AI) and point-of-care ultrasound.

Recent studies have highlighted the prognostic value of right ventricular (RV) longitudinal strain, RV end-systolic dimensions, and right atrial (RA) size and function in pulmonary hypertension and heart failure. While RA pressure is a central marker of right heart diastolic function, the recent emphasis on venous excess imaging (VExUS) has provided granularity to the systemic consequences of RH failure. Several methodological advances are also changing the landscape of RH imaging including post-processing 3D software to delineate the non-longitudinal (radial, anteroposterior, and circumferential) components of RV function, as well as AI segmentation- and non-segmentation-based quantification. Together with recent guidelines and advances in AI technology, the field is shifting from specific RV functional metrics to integrated RH disease-specific phenotypes. A modern echocardiographic evaluation of RH function should focus on the entire cardiopulmonary venous unit-from the venous to the pulmonary arterial system. Together, a multi-parametric approach, guided by physiology and AI algorithms, will help define novel integrated RH profiles for improved disease detection and monitoring.

Abnormal septal motion (ASM), which often is associated with myocardial ischemia, is also observed in other diseases. Owing to the position of the interventricular septum (IVS) in the heart, its movement not only relies on contractile properties but is also affected by the pressure gradient between the 2 ventricles and by the mode of electrical activation. Echocardiography allows the operator to focus on the motion of the IVS, analyzing its characteristics and thereby gaining information about the possible underlying pathophysiological mechanism. In this review, we focused on the main echocardiographic patterns of ASM that are not related to a failure of contractile properties of the septum (i.e., acute coronary syndrome and cardiomyopathies), showing their pathophysiological mechanisms and underlining their diagnostic usefulness in clinical practice.

The Quality Enhancement Research Initiative (QuERI) in adults with congenital heart disease (ACHD) was developed to improve detection of pulmonary arterial hypertension (PAH) after repair of systemic-to-pulmonary arterial shunt lesions.

This study sought to standardize use of accepted criteria for PAH diagnosis and evaluate utility in at-risk patients with ACHD.

Patients ≥18 years of age with ACHD repaired ≥1 year before enrollment and with additional risk factors for developing PAH were eligible. History, physical examination, electrocardiogram, transthoracic echocardiogram, World Health Organization functional class, and 6-minute walk distance were evaluated at baseline and yearly for 3 years. Pop-up reminders of patient-specific evidence-based recommendations for PAH detection appeared during data entry.

Among 217 eligible patients, mean age (enrollment) was 44.0 ± 15.9 years, 72.3% were women, and 82.0% were World Health Organization functional class I. Electrocardiogram was performed in >80% and TTE in >70% of patients annually; capture of required transthoracic echocardiography (TTE) measures and alignment between study- and core-center interpretation improved over time, with more frequent assessment of pulmonary arterial flow acceleration time and documentation of right ventricular outflow tract Doppler notching. Approximately 40% of patients had ≥2 high-risk features for PAH on TTE, but only 7% (6/82) underwent right heart catheterization (RHC). Using current definitions, 2 patients were confirmed by RHC to have a diagnosis of PAH (maximum follow-up 3 years).

A structured protocol may improve screening for patients with repaired ACHD at risk of developing PAH. RHC may be underutilized in patients with ACHD with TTE findings suggestive of PAH. (Adult Congenital Heart Disease Registry [QuERI]; NCT01659411).

Pulmonary embolism (PE) and pulmonary hypertension (PH) are potentially fatal disease states. Early diagnosis and goal-directed management improve outcomes and survival. Both conditions share several echocardiographic findings of right ventricular dysfunction. This can inadvertently lead to incorrect diagnosis, inappropriate and potentially harmful management, and delay in time-sensitive therapies. Fortunately, bedside echocardiography imparts a few critical distinctions.

This narrative review describes eight physiologically interdependent echocardiographic parameters that help distinguish acute PE and chronic PH. The manuscript details each finding along with associated pathophysiology and summarization of the literature evaluating diagnostic utility. This guide then provides pearls and pitfalls with high-quality media for the bedside evaluation.

The echocardiographic parameters suggesting acute or chronic right ventricular dysfunction (best used in combination) are: 1. Right heart thrombus (acute PE) 2. Right ventricular free wall thickness (acute ≤ 5 mm, chronic > 5 mm) 3. Tricuspid regurgitation pressure gradient (acute ≤ 46 mmHg, chronic > 46 mmHg, corresponding to tricuspid regurgitation maximal velocity ≤ 3.4 m/sec and > 3.4 m/sec, respectively) 4. Pulmonary artery acceleration time (acute ≤ 60-80 msec, chronic < 105 msec) 5. 60/60 sign (acute) 6. Pulmonary artery early-systolic notching (proximally-located, higher-risk PE) 7. McConnell's sign (acute) 8. Right atrial enlargement (equal to left atrial size suggests acute, greater than left atrial size suggests chronic).

Emergency physicians must appreciate the echocardiographic findings and associated pathophysiology that help distinguish acute and chronic right ventricular dysfunction. In the proper clinical context, these findings can point towards PE or PH, thereby leading to earlier goal-directed management.

Echocardiographic diagnosis of pulmonary hypertension (PH) in horses is usually based on Doppler interrogation of tricuspid (TR) or pulmonic regurgitation (PR). This study aimed at investigating two-dimensional echocardiographic (2DE) variables, including pulmonary artery diameter (PAD) and pulmonary artery distensibility index (PADI), to diagnose PH.

41 healthy horses; 82 horses with TR or PR and normal intracardiac pressure gradients; and 35 horses with TR or PR velocities indicating PH. The 2DE variables were compared between groups, cut-offs for the diagnosis of PH were determined, and conditional inference trees served to identify the variable best predicting PH in the absence of TR or PR.

Horses with PH had larger end-diastolic cross-sectional (short-axis) PAD (PADed-sx) (5.8 ± 1.0 cm; mean ± SD) and smaller PADI[/PADps] (15.4 ± 7.7%) than healthy horses (5.0 ± 0.6 cm, P<0.0001; 20.5 ± 4.4%, P<0.001) and horses with TR or PR but no PH (5.3 ± 0.6 cm, P=0.003; 18.8 ± 4.2%, P=0.005). PADed-sx predicted PH with sensitivity (Se) = 36% and specificity (Sp) = 94% (cut-off 6.0 cm, AUC = 0.668, P=0.002), while PADI[/PADps] predicted PH with Se = 43% and Sp = 92% (cut-off 13.7%, AUC = 0.662, P=0.004). In the absence of TR, PADed-sx and the end-diastolic long-axis aortic diameter-to-PADed-sx ratio (AoDed-lx/PADed-sx) were most suitable to diagnose PH.

Pulmonary artery crosssectional (short-axis) diameter at end-diastole measured in a right-parasternal long axis view of the left ventricular outflow tract and Pulmonary artery distensibility index were moderately specific but not very sensitive to diagnosing PH. Nonetheless, they may be used as complementary indices suggesting PH in the absence of TR or PR.

The aim was to evaluate the relationship between gut dysbiosis and hemodynamic changes (hyperdynamic circulation) in cirrhosis, and between hemodynamic changes and complications of this disease. This study included 47 patients with cirrhosis. Stool microbiome was assessed using 16S rRNA gene sequencing. Echocardiography with a simultaneous assessment of blood pressure and heart rate was performed to assess systemic hemodynamics. Patients with hyperdynamic circulation had more severe cirrhosis, lower albumin, sodium and prothrombin levels, higher C-reactive protein, aspartate aminotransferase and total bilirubin levels, and higher incidences of portopulmonary hypertension, ascites, overt hepatic encephalopathy, hypoalbuminemia, hypoprothrombinemia, systemic inflammation, and severe hyperbilirubinemia than patients with normodynamic circulation. Patients with hyperdynamic circulation compared with those with normodynamic circulation had increased abundance of Proteobacteria, Enterobacteriaceae, Bacilli, Streptococcaceae, Lactobacillaceae, Fusobacteria, Micrococcaceae, Intestinobacter, Clostridium sensu stricto, Proteus and Rumicoccus, and decreased abundance of Bacteroidetes, Bacteroidaceae, Holdemanella, and Butyrivibrio. The systemic vascular resistance and cardiac output values correlated with the abundance of Proteobacteria, Enterobacteriaceae, Bacilli, Streptococcaceae, Lactobacillaceae, Micrococcaceae, and Fusobacteria. Heart rate and cardiac output value were negatively correlated with the abundance of Bacteroidetes. The mean pulmonary artery pressure value was positively correlated with the abundance of Proteobacteria and Micrococcaceae, and negatively with the abundance of Holdemanella.

The classification and management of pulmonary hypertension (PH) is challenging due to clinical heterogeneity of patients. We sought to identify distinct multimorbid phenogroups of patients with PH that are at particularly high-risk for adverse events.

A hospital-based cohort of patients referred for right heart catheterization between 2005-2016 with PH were included. Key exclusion criteria were shock, cardiac arrest, cardiac transplant, or valvular surgery. K-prototypes was used to cluster patients into phenogroups based on 12 clinical covariates.

Among 5208 patients with mean age 64±12 years, 39% women, we identified 5 distinct multimorbid PH phenogroups with similar hemodynamic measures yet differing clinical outcomes: (1) "young men with obesity", (2) "women with hypertension", (3) "men with overweight", (4) "men with cardiometabolic and cardiovascular disease", and (5) "men with structural heart disease and atrial fibrillation." Over a median follow-up of 6.3 years, we observed 2182 deaths and 2002 major cardiovascular events (MACE). In age- and sex-adjusted analyses, phenogroups 4 and 5 had higher risk of MACE (HR 1.68, 95% CI 1.41-2.00 and HR 1.52, 95% CI 1.24-1.87, respectively, compared to the lowest risk phenogroup 1). Phenogroup 4 had the highest risk of mortality (HR 1.26, 95% CI 1.04-1.52, relative to phenogroup 1).

Cluster-based analyses identify patients with PH and specific comorbid cardiometabolic and cardiovascular disease burden that are at highest risk for adverse clinical outcomes. Interestingly, cardiopulmonary hemodynamics were similar across phenogroups, highlighting the importance of multimorbidity on clinical trajectory. Further studies are needed to better understand comorbid heterogeneity among patients with PH.

The role of echocardiography in the diagnostic and prognostic assessment of pulmonary hypertension (PH) has been widely studied recently. However, these findings have not undergone normative evaluation and may provide confusing evidence for clinicians. To evaluate and summarize existing evidence, we performed an umbrella review.

Systematic reviews and meta-analyses were searched in PubMed, Embase, Web of Science, and Cochrane Library from inception to September 4, 2022. The methodological quality of the included studies was assessed using Assessment of Multiple Systematic Reviews (AMSTAR), and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system was used to evaluate the quality of evidence.

Thirteen meta-analyses (nine diagnostic and four prognostic studies) were included after searching four databases. The methodological quality of the included studies was rated as high (62%) or moderate (38%) by AMSTAR. The thirteen included meta-analyses involved a total of 28 outcome measures. The quality of evidence for these outcomes were high (7%), moderate (29%), low (39%), and very low (25%) using GRADE methodology. In the detection of PH, the sensitivity of systolic pulmonary arterial pressure is 0.85-0.88, and the sensitivity and specificity of right ventricular outflow tract acceleration time are 0.84. Pericardial effusion, right atrial area, and tricuspid annulus systolic displacement provide prognostic value in patients with pulmonary arterial hypertension with hazard ratios between 1.45 and 1.70. Meanwhile, right ventricular longitudinal strain has independent prognostic value in patients with PH, with a hazard ratio of 2.96-3.67.

The umbrella review recommends echocardiography for PH detection and prognosis. Systolic pulmonary arterial pressure and right ventricular outflow tract acceleration time can be utilized for detection, while several factors including pericardial effusion, right atrial area, tricuspid annular systolic displacement, and right ventricular longitudinal strain have demonstrated prognostic significance.

PROSPERO (CRD42022356091), https://www.crd.york.ac.uk/prospero/ .

Pulmonary arterial hypertension (PAH) is a severe, high mortality and progressive disease. Early diagnosis and treatment improves the prognosis. Patients with scleroderma disease presents high risk of developing PAH. Established screening strategies - echocardiogram and DETECT algorithm - recognize the disease when it is already advanced. Cardiopulmonary exercise testing (CPET) detects pulmonary vascular injury in earlier stages.

Prospective study of 52 consecutive patients diagnosed of scleroderma in our health area, during 2 years (2018 and 2019). All of them undergo CPET, in addition to the annual systematic screening. Sensitivity of current PAH screening is compared to CPET. To confirm the presence of PAH, right heart catheterization (RHC) is performed. In case of suspected PAH in CPET, but non-confirmatory right heart catheterization at rest, patients carried out exercise RHC.

Fifty-two CPET were performed, of which 16 suggested PAH. Resting RHC confirmed PAH in 5 patients and exercise RHC in 7 (diagnostic sensitivity of CPET together with rest and exercise catheterization of 100%). Of these 16 patients, DETECT had identified 10, of whom resting RHC confirmed PAH in 3 and exercise RHC in 2 (guideline-based diagnostic algorithm sensitivity 70%).

CPET and exercise RHC could detect PAH earlier than established screening in patients with scleroderma disease, allowing early diagnosis.

Right ventricular-pulmonary artery coupling (RV-PA coupling) refers to the relationship between RV contractility and RV afterload. Normal RV-PA coupling is maintained only when RV function and pulmonary vascular resistance are appropriately matched. RV-PA uncoupling occurs when RV contractility cannot increase to match RV afterload, resulting in RV dysfunction and right heart failure. RV-PA coupling plays an important role in the pathophysiology and progression of cardiovascular diseases. Therefore, early and accurate evaluation of RV-PA coupling is of great significance for a patient's condition assessment, clinical decision making, risk stratification, and prognosis judgment. RV-PA coupling can be assessed by using invasive or noninvasive approaches. The aim of this review was to summarize the pathological mechanism and evaluation methods of RV-PA coupling, the advantages and disadvantages of each method, and the application value of RV-PA coupling in various cardiovascular diseases.