Search

Background: Pulmonary arterial hypertension (PAH) is a severe disease characterized by elevated blood pressure in the pulmonary artery that can ultimately damage the right ventricle of the heart. PAH is pathophysiologically heterogeneous, which makes early diagnosis and treatment difficult. Inflammation is thought to be an important factor in the development and progression of this disease and may explain some of the observed interindividual differences. In the context of both acute and chronic inflammation, neutrophil recruitment to the lung has been suggested as a potential biomarker for studying PAH progression. However, there are currently no specific probes for its non-invasive in vivo detection. The imaging-based gold standard for assessing inflammation is [ 18 F] fluorodeoxyglucose ( 18 F-FDG), which is not cell specific. This highlights the urgent need for more specific molecular probes to support personalized medicine., Methods: This study investigated the potential of magnetic nanoradiotracers based on ultrasmall iron oxide nanoparticles, functionalized with N-cinnamoyl-F-(D)L-F-(D)L-F peptide, to detect increased neutrophil infiltration in vivo in different PAH animal models via positron emission tomography. These nanoprobes target formyl peptide receptor 1, which is abundantly expressed in the cell membrane of neutrophils. To assess the benefit of these nanoprobes, their biodistribution was first assessed via magnetic resonance imaging and histology. Then, their lung uptake was compared by positron emission tomography with that of 18 F-FDG in two types of PAH animal models with different profiles of inflammation and neutrophil infiltration: monocrotaline and double-hit Sugen-chronic hypoxia PAH rat models., Results: Our targeted magnetic nanoradiotracer detected an increase in pulmonary neutrophil infiltration in both PAH models and distinguished between them, which was not possible with 18 F-FDG PET., Conclusions: This study underscores the importance of targeted imaging in providing an individualized and longitudinal evaluation of heterogeneous and multifactorial diseases such as PAH. The use of targeted multimodal nanoprobes, for magnetic resonance/positron emission tomography imaging has the potential to facilitate the diagnosis and monitoring of diseases, as well as the development of novel therapies., Competing Interests: Declarations Ethics approval and consent to participate Animal handling was conducted under the European Council Directive 2010/63/UE. The Ethical Committee at CIC biomaGUNE and local authorities Diputación Foral de Guipuzcoa (project authorization numbers: PRO-AE-SS-225 and PRO-AE-SS-112) approved all the animal experiments. Consent for publication We acknowledge that the manuscript text and any associated images submitted for publication in the journal will be made freely accessible on the internet, available for view by the general public. Competing interests The authors declare no competing interests., (© 2024. The Author(s).)

Aims: Novel echocardiographic parameters of right ventricular (RV) function, including speckle-tracking-derived, three-dimensional, and RV-pulmonary artery coupling parameters, have emerged for the evaluation of pulmonary arterial hypertension (PAH). The relative role of these parameters in the risk stratification of PAH patients is unclear. We compared the performance of multiple RV parameters and sought to establish an optimal model for identifying the risk profile of patients with PAH., Methods and Results: Comprehensive risk assessments were performed for 70 patients with PAH. The risk profile of every patient was determined based on the guideline recommendations. Conventional parameters, including fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE), novel speckle-tracking-derived RV longitudinal strain (RVLS), and three-dimensional RV ejection fraction (3D-RVEF), were used to evaluate RV function. Pressure-strain loops were measured for the assessment of RV myocardial work, including RV global wasted work (RVGWW). RV-pulmonary artery coupling was assessed by indexing RV parameters to the estimated pulmonary artery systolic pressure (PASP). The median age was 34 (30-43) years, and 62 (88.6%) patients were female. Forty-five patients were classified into the low-risk group, while 25 patients were classified into the intermediate-high-risk group. Most RV parameters could be used to determine the risk profile and exhibited significantly improved diagnostic performance after indexing to PASP (including FAC/PASP, TAPSE/PASP, and 3D-RVEF/PASP). RVLS/PASP showed the best performance, with an area under the curve of 0.895. In multivariate analysis (Model 1), only RVGWW (>90.5 mmHg%), RVLS (> -16.7%), and TAPSE (<17.5 mm) remained significant (all P < 0.05). Model 1 outperformed every single RV parameter, with a significantly larger area under the curve (all P < 0.05). With PASP indexing in Model 2, RVLS/PASP > -0.275 [odds ratio (OR) 20.63, 95% confidence interval (CI) 4.62-92.11, P < 0.001] and RVGWW > 90.5 mmHg% (OR 6.17, 95% CI 1.37-27.76, P = 0.018) independently identified a higher risk profile. The addition of RVGWW to two models determined incremental value in identification (continuous net reclassification improvement 1.058, 95% CI 0.639-1.477, P < 0.001)., Conclusions: The combination models for RV function outperformed any single parameter in identifying the risk profile of patients with PAH. Comprehensive assessment of RV-pulmonary artery coupling using multiparametric methods is clinically meaningful in patients with PAH., (© 2024 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Introduction: Accurate identification of pulmonary arterial hypertension (PAH) in primary care and rural areas can be a challenging task. However, recent advancements in computer vision offer the potential for automated systems to detect PAH from echocardiography., Objectives: Our aim was to develop a precise and efficient diagnostic model for PAH tailored to the unique requirements of intelligent diagnosis, especially in challenging locales like high-altitude regions., Methods: We proposed the Chamber Attention Network (CAN) for PAH identification from echocardiographic images, trained on a dataset comprising 13,912 individual subjects. A convolutional neural network (CNN) for view classification was used to select the clinically relevant apical four chamber (A4C) and parasternal long axis (PLAX) views for PAH diagnosis. To assess the importance of different heart chambers in PAH diagnosis, we developed a novel Chamber Attention Module., Results: The experimental results demonstrated that: 1) The substantial correspondence between our obtained chamber attention vector and clinical expertise suggested that our model was highly interpretable, potentially uncovering diagnostic insights overlooked by the clinical community. 2) The proposed CAN model exhibited superior image-level accuracy and faster convergence on the internal validation dataset compared to the other four models. Furthermore, our CAN model outperformed the others on the external test dataset, with image-level accuracies of 82.53% and 83.32% for A4C and PLAX, respectively. 3) Implementation of the voting strategy notably enhanced the positive predictive value (PPV) and negative predictive value (NPV) of individual-level classification results, enhancing the reliability of our classification outcomes., Conclusions: These findings indicate that CAN is a feasible technique for AI-assisted PAH diagnosis, providing new insights into cardiac structural changes observed in echocardiography., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Production and hosting by Elsevier B.V.)

Objectives: Right ventricular (RV) output reserve, defined as increase of cardiac output during exercise, is reduced in patients with pulmonary arterial hypertension (PAH). Aim of this study was to evaluate the association of right heart size measured by echocardiography and invasively measured RV function at rest and during exercise in PAH patients., Methods: Adult PAH-patients who received routine haemodynamic assessment at rest and during exercise by right heart catheterisation and echocardiographic measurement of right heart size (right atrial (RA) and RV area) were included in this study. Clinical, echocardiographic, laboratory, exercise and invasive haemodynamic parameters were retrospectively analysed. The primary endpoint was to assess the association between right heart size and right ventricular function., Results: Data from 215 PAH patients (age 58.9 ± 15.9 years, 63.3% female, 62.2% double or triple combination treatment) were analysed in this cross-sectional study. Cardiac index was significantly lower for patients with enlarged RA-area > 18 cm 2 at rest, and at 25 and 50 W (all p < 0.001) and for patients with enlarged RV area > 20 cm 2 at rest, 25, 50 and 75 W (all p < 0.001). Furthermore, pulmonary vascular resistance and mPAP/CO slope (all p < 0.001) were significantly higher and pulmonary arterial compliance (all p < 0.05) was significantly lower in patients with enlarged RA or RV area. RA and RV area correlated with TAPSE/sPAP (both p < 0.001, R - 0.570 and - 0.530)., Conclusion: This study could underline that an enlargement of RA- and RV-area is associated with an impaired RV function at rest and during exercise in patients with PAH., Competing Interests: Declaration of competing interest BE: received travel fees, consulting fees, speaking fees, and/or honoraria from Janssen, MSD, Bayer and OMT, outside the submitted work. KFW, AMM: nothing to declare. CAE: received speaking fees from MSD outside the submitted work. SH: received honoraria for lectures, consultancy, or both from Actelion, AOP, Bayer/MSD, GSK, Novartis, OMT, Pfizer, and United Therapeutics out-side the submitted work. EG: has received grants and personal fees from Actelion, Bayer AG, and MSD; grants from GSK, Novartis, and United Therapeutics and personal fees from SCOPE, OrPha Swiss GmbH, and Zurich Heart House, outside the submitted work. PX: has received personal fees from MSD and OMT outside the submitted work. NB: received speaker fees from University Hospital Zurich and AOP., (Copyright © 2024. Published by Elsevier B.V.)

Pulmonary arterial hypertension (PAH) has a subtle onset, rapid progression, and high mortality rate. Imaging evaluation is an important diagnostic and follow-up method for PAH patients. Right ventricular (RV) strain evaluation can identify early changes in RV function and predict the prognosis. Currently, various methods such as tissue Doppler imaging, velocity vector imaging, speckle tracking imaging, and cardiac magnetic resonance imaging can be used to evaluate RV strain in PAH patients. This article aims to summarize the research progress of RV strain imaging evaluation technology in PAH patients, in order to provide a basis for clinical diagnosis and follow-up of PAH patients.

Objectives: Bosentan is a dual endothelin receptor antagonist approved for the treatment of SSc digital ulcers (DU) and pulmonary arterial hypertension (PAH). Systolic pulmonary arterial pressure (sPAP) is a relevant parameter for the follow-up and prognosis of SSc-PAH. The therapeutic magnitude of bosentan in SSc-PAH is not fully understood, thus we aim to establish the degree of sPAP reduction in bosentan treated SSc-PAH patients., Methods: We performed a systematic literature review in three databases from January 2000 to June 2023, involving sPAP measurement at transthoracic echocardiography of SSc patients before and after starting bosentan. Following the study quality assessment and data extraction, we performed random-effects meta-analysis and Egger's test for publication bias. Stratified analysis was performed for mono-/combination therapy, follow up duration (≤1 year), indication for bosentan therapy (PAH or DU/mixed)., Results: In the 11 selected manuscripts, sPAP mean difference before and after bosentan therapy was - 5.63mmHg (CI95% -9.79 to -1.48, p=0.0078). In stratified analysis, sPAP mean was significantly different before and after bosentan therapy only for studies considering < 1 year of follow-up (p=0.0020), monotherapy (p=0.0140) and the strict indication for PAH (p=0.0002)., Conclusions: Bosentan significantly decreases sPAP, a relevant prognostic marker, especially in overt SSc-PAH. However, bosentan did not decrease sPAP when started for DU/mixed indication nor for follow-up>1 year. The burden of publication bias was significant. Therefore, further studies are required to assess bosentan's haemodynamic effect in high-risk patients for SSc-PAH.

The pathophysiology underlying impaired LV function in PAH remains unclear, with some studies implicating intrinsic myocardial dysfunction and others pointing to LV underfilling. Evaluation of pulmonary vein area (PVA) and flow may offer novel, mechanistic insight by distinguishing elevated LV filling pressure common in myocardial dysfunction from LV underfilling. This study aimed to elucidate LV filling physiology in PAH by assessing PVA and flow using cardiac magnetic resonance (CMR) and compare pulmonary vein flow in PAH with HFrEF as a model representing elevated filling pressures, in addition to healthy controls. Patients with PAH or heart failure with reduced ejection fraction (HFrEF) referred for CMR were retrospectively reviewed, and healthy controls were included as reference. Pulmonary vein S, D and A-wave were compared between groups. Associations between pulmonary vein area (PVA) by CMR and echocardiographic indices of LV filling pressure were evaluated. Nineteen patients with PAH, 25 with HFrEF and 24 controls were included. Both PAH and HFrEF had lower ejection fraction and S-wave velocity than controls. PAH displayed smaller LV end-diastolic volumes than controls, while HFrEF demonstrated larger PVA and higher A-wave reversal. PVA was associated with mitral E/e' ratio (r 2  = 0.10; p = 0.03), e' velocity (r 2  = 0.23; p = 0.001) and left atrial volume (r 2  = 0.07; p = 0.005). Among PAH, PVA was not associated with LV-GLS. A PVA cut-off of 2.3cm 2 displayed 87% sensitivity and 72% specificity to differentiate HFrEF and PAH (AUC = 0.82). PAH displayed lower pulmonary vein S-wave velocity, smaller LV volume and reduced function compared with controls. Reduced LV function in PAH may be owing to underfilling rather than intrinsic myocardial disease. PVA demonstrates promise as a novel, non-invasive imaging marker to assess LV filling status., (© 2024. The Author(s).)

Background: The risk stratification of pulmonary arterial hypertension proposed by the European Society of Cardiology /European Respiratory Society guidelines in 2015 and 2022 included two to three echocardiographic indicators. However, the specific value of echocardiography in risk stratification of pre-capillary pulmonary hypertension (pcPH) has not been efficiently demonstrated. Given the complex geometry of the right ventricular (RV) and influencing factors of echocardiographic parameter, there is no single echocardiographic parameter that reliably informs about PH status. We hypothesize that a multi-parameter comprehensive index can more accurately evaluate the severity of the pcPH. The purpose of this study was to develop and validate an echocardiographic risk score model to better assist clinical identifying high risk of pcPH during initial diagnosis and follow-up., Methods: We studied 197 consecutive patients with pcPH. A multivariable echocardiographic model was constructed to predict the high risk of pcPH in the training set. Points were assigned to significant risk factors in the final model based on β-coefficients. We validated the model internally and externally., Results: The echocardiographic score was constructed by multivariable logistic regression, which showed that pericardial effusion, right atrial (RA) area, RV outflow tract proximal diameter (RVOT-Prox), the velocity time integral of the right ventricular outflow tract (TVI RVOT ) and S' were predictors of high risk of pcPH. The area under curve (AUC) of the training set of the scoring model was 0.882 (95%CI: 0.809-0.956, p < 0.0001). External validation was tested in a test dataset of 77 patients. The AUC of the external validation set was 0.852. A 10-point score risk score was generated, with scores ranging from 0 to 10 in the training cohort. The estimate risk of high risk of pcPH ranged from 25.1 to 94.6%., Conclusions: The echocardiographic risk score using five echocardiographic parameters could be comprehensive and useful to predict the high risk of pcPH for initial assessment and follow-up., (© 2024. The Author(s).)

Reduced pulmonary diffusing capacity for carbon monoxide (D LCO ) can be observed in pulmonary arterial hypertension (PAH) and associates with increased mortality. However, the prognostic value of D LCO when corrected for haemoglobin (D LCOc ), an independent modifier of D LCO , remains understudied. Additionally, the prognostic role of ventilation (V)-perfusion (Q) emission computed tomography (V/Q SPECT) findings in patients with PAH, which may concurrently be performed to rule out chronic thromboembolic pulmonary hypertension, is uncertain. A retrospective cohort study was conducted on 152 patients with PAH referred to a tertiary hospital for evaluation from January 2011 to January 2020. Lung function tests, clinical data and V/Q SPECT were ascertained. Cox regression analysis was performed to evaluate the association between D LCOc , D LCO and V/Q SPECT defects at referral with all-cause mortality. In equally adjusted Cox regression analysis, each percentage increase in D LCOc % predicted (%pred) (hazard ratio (HR) 0.97; 95% CI: 0.94-0.99) and D LCO %pred (HR 0.97; 95% CI: 0.94-0.99) was similarly associated with all-cause mortality. There was no detectable difference in area under the curve for prediction of all-cause mortality by D LCOc %pred and D LCO %pred (C-index 0.71 and 0.72, respectively, P = 0.85 for difference). None of the defects noted on V/Q SPECT were significantly associated with mortality, but mismatched defects were associated with lower values of D LCOc %pred and D LCO %pred. D LCOc %pred and D LCO %pred perform equally as prognostic markers in PAH, supporting the use of either metric when available for prognostic stratification., (© 2024 The Authors. Experimental Physiology published by John Wiley & Sons Ltd on behalf of The Physiological Society.)

Purpose: The purpose of this study was to evaluate the accuracy of four-dimensional flow cardiac magnetic resonance imaging (4D flow MRI) compared to right heart catheterization in measuring pulmonary flow (Qp), systemic flow (Qs) and pulmonary-to-systemic flow ratio (Qp/Qs) in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD)., Materials and Methods: The study was registered on Clinical-trial.gov (NCT03928002). Sixty-four patients with PAH-CHD who underwent 4D flow MRI were included. There were 16 men and 48 women with a mean age of 45.3 ± 13.7 (standard deviation [SD]) years (age range: 21-77 years). Fifty patients (50/64; 78%) presented with pre-tricuspid shunt. Qp (L/min), Qs (L/min) and Qp/Qs were measured invasively using direct Fick method during right heart catheterization and compared with measurements assessed by 4D flow MRI within a 24-48-hour window., Results: The average mean pulmonary artery pressure was 51 ± 17 (SD) mm Hg with median pulmonary vascular resistance of 8.8 Wood units (Q1, Q3: 5.3, 11.7). A strong linear correlation was found between Qp measurements obtained with 4D flow MRI and those obtained with the Fick method (r = 0.96; P < 0.001). Bland Altman analysis indicated a mean difference of 0.15 ± 0.48 (SD) L/min between Qp estimated by 4D flow MRI and by right heart catheterization. A strong correlation was found between Qs and Qp/Qs measured by 4D flow MRI and those obtained with the direct Fick method (r = 0.85 and r = 0.92; P < 0.001 for both)., Conclusion: Qp as measured by 4D flow MRI shows a strong correlation with measurements derived from the direct Fick method. Further investigation is needed to develop less complex and standardized methods for measuring essential PAH parameters, such as pulmonary arterial pressures and pulmonary vascular resistance., Competing Interests: Declaration of competing interest The authors declare no actual or potential conflict of interest related to this study., (Copyright © 2024 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

Background: For patients with congenital heart disease-related pulmonary arterial hypertension (CHD-PAH), cardiopulmonary exercise testing (CPET) can reflect cardiopulmonary reserve function. However, CPET may not be readily accessible for patients with high-risk conditions or limited mobility due to disability. Echocardiography, on the other hand, serves as a widely available diagnostic tool for all CHD-PAH patients. This study was aimed to identify the parameters of echocardiography that could serve as indicators of cardiopulmonary function and exercise capacity., Methods: A cohort of 70 patients contributed a total of 110 paired echocardiogram and CPET results to this study, with 1 year interval for repeated examinations. Echocardiography and exercise testing were conducted following standardized procedures, and the data were collected together with clinically relevant indicators for subsequent statistical analysis. Demographic comparisons were performed using t-tests and chi-square tests. Univariate and multivariate analyses were conducted to identify potential predictors of peak oxygen uptake (peak VO 2 ) and the carbon dioxide ventilation equivalent slope (VE/VCO 2 slope). Receiver operating characteristic (ROC) analysis was used to assess the performance of the parameters., Results: The ratio of tricuspid annular plane systolic excursion to pulmonary artery systolic pressure (TAPSE/PASP) was found to be the only independent indicator significantly associated with both peak VO 2 and VE/VCO 2 slope (both p < 0.05). Additionally, left ventricular ejection fraction (LVEF) and right ventricular fractional area change (FAC) were independently correlated with the VE/VCO 2 slope (both p < 0.05). TAPSE/PASP showed the highest area under the ROC curve (AUC) for predicting both a peak VO 2  ≤ 15 mL/kg/min and a VE/VCO 2 slope ≥ 36 (AUC = 0.91, AUC = 0.90, respectively). The sensitivity and specificity of TAPSE/PASP at the optimal threshold exceeded 0.85 for both parameters., Conclusions: TAPSE/PASP may be a feasible echocardiographic indicator for evaluating exercise tolerance., (© 2024. The Author(s).)

Background: Atrial septal defects (ASD) are the most common type of adult congenital heart disease (ACHD) associated with a high risk developing of pulmonary arterial hypertension (PAH). ASD closure is not recommended in patients with PAH and Pulmonary Vascular Resistance (PVR) ≥ 5 Wood Unit (WU). Noninvasive methods have been proposed to measure PVR; however, their accuracy remains low. Right Ventricle (RV) - Pulmonary Artery (PA) coupling is defined as the ability of the RV to adapt to high-resistance conditions. Tricuspid Annular Plane Systolic Excursion (TAPSE)/estimated pulmonary artery systolic pressure (ePASP) calculation using echocardiography is a noninvasive technique that has been proposed as a surrogate equation to evaluate RV-PA coupling. Currently, no research has demonstrated a relationship between RV-PA coupling and PVR in patients with ASD., Methods: The study participants were consecutive eligible patients with ASD who underwent right heart catheterization (RHC) and echocardiography at Hasan Sadikin General Hospital, Bandung. Both the procedures were performed on the same day. RV-PA Coupling, defined as TAPSE/ePASP > 0.31, was assessed using echocardiography. The PVR was calculated during RHC using the indirect Fick method., Results: There were 58 patients with ASD underwent RHC and echocardiography. Among them, 18 had RV/PA Coupling and 40 had RV/PA Uncoupling. The PVR values were significantly different between the two groups (p = 0.000). Correlation test between TAPSE/ePASP with PVR showed moderate negative correlation (r= -0.502, p = 0.001). TAPSE/ePASP ≤ 0.34 is the cutoff point to predict PVR > 5 WU with sensitivity of 91.7% and specificity 63.6%., Conclusion: This study showed a moderate negative correlation between TAPSE/ePASP and PVR. TAPSE/ePASP ≤ 0.34 could predict PVR > 5 WU with good sensitivity., (© 2024. The Author(s).)

Objective: To evaluate differences in nailfold videocapillaroscopy (NVC) findings between SSc patients with and without a diagnosis of pulmonary arterial hypertension (PAH)., Methods: One hundred and ten SSc patients were enrolled in this cross-sectional, case-control, multicentre study. Patients were divided into cases (SSc-PAH confirmed by right heart catheterization) and controls (SSc-nonPAH with low probability of PAH). NVC patterns (early, active and late) and morphological parameters (microvascular density, non-specific abnormalities, giant capillaries, micro-haemorrhages, avascular areas) were considered using a semiquantitative scoring system., Results: SSc-PAH patients showed higher frequencies of late pattern (P < 0.01), non-specific abnormalities (P < 0.01), lower capillary density (P < 0.01), higher avascular areas (P < 0.01) and a higher mean NVC score (P < 0.01). Contrarily, the early/active pattern (P < 0.01) and a higher rate of micro-haemorrhages (P = 0.04) were more frequent in non-PAH patients. By a multivariate analysis, SSc-PAH patients, compared with non-PAH, had more non-specific abnormalities [27/55, 49.1% vs 10/55, 18.2%; adjusted odd ratio (OR) 16.89; 95% CI: 3.06, 93.16], a lower capillary density (grade 3, 20/55, 36.4% vs 5/55, 9.1%; adjusted OR 38.33; 95% CI: 2.34, 367.80) and avascular areas (18/55, 32.7% vs 10/55, 18.2%; adjusted OR 16.90; 95% CI: 2.64, 44.35). A correlation was found between the mean pulmonary arterial pressure and avascular areas (P < 0.01), capillary density (P < 0.01) and non-specific abnormalities (P < 0.01). A clinical model including the NVC variables may be able to predict a diagnosis of PAH., Conclusion: Our results indicate that the distinctive peripheral microcirculatory injury of SSc, i.e. capillary loss and morphological abnormalities, appear more severe and pronounced in patients with SSc-PAH., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Pulmonary arterial hypertension (PAH) is a common complication of systemic lupus erythematosus (SLE), and PAH can cause right ventricle (RV) remodel and dyssynchrony. The aim of this study was to explore the value of RV dyssynchrony in predicting adverse clinical events in patients with systemic lupus erythematosus-aaociated pulmonary arterial hypertension (SLE-PAH) using two-dimensional speckle tracking echocardiography (2D-STE). A total of 53 patients with SLE-PAH were enrolled in this study. The dyssynchrony of the RV (RV-SD6) was evaluated by 2D-STE. The clinical data of all participants were collected, and routine cardiac function parameters were measured by two-dimensional echocardiography, and analyzed for their correlation with RV-SD6. The predictive value of RV-SD6 in clinical adverse event was evaluated. RV-SD6 was negatively correlated with RV-FLS, RV-FAC, and TAPSE (r = - 0.788, r = - 0.363 and r = - 0.325, respectively, all P < 0.01), while the correlation with RV-FLS was the strongest. linear regression analysis showed that RV-FLS was an independent risk factor for RV-SD6 (β = - 1.40, 95% CI - 1.65 ~ - 1.14, P < 0.001). Cox regression analysis showed that RV-SD6 was a predictor with clinical adverse events (HR = 1.03, 95% CI 1 ~ 1.06, P < 0.05). RV-SD6 was highly discriminative in predicting clinical adverse events (AUC = 0.764), at a cutoff of 51.10 ms with a sensitivity of 83.3% and specificity of 68.3%. RV-FLS was negatively correlated with RV-SD6 and was an independent risk factor for it. RV-SD6 can serve as an indicator for predicting the occurrence of adverse clinical events in SLE-PAH patients, with high sensitivity and specificity., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Aims: Systemic sclerosis (SSc) is characterized by vasculopathy, inflammation, and fibrosis, and carries one of the worst prognoses if patients also develop pulmonary arterial hypertension (PAH). Although PAH is a known prognosticator, patients with SSc-PAH demonstrate disproportionately high mortality, presumably due to cardiac involvement. In this cross-sectional study, the relationship between cardiac involvement revealed by cardiovascular magnetic resonance (CMR) and systemic microvascular disease severity measured with nailfold capillaromicroscopy (NCM) in patients with SSc-PAH is evaluated and compared with patients with idiopathic PAH (IPAH)., Methods and Results: Patients with SSc-PAH and IPAH underwent CMR, echocardiography, and NCM with post-occlusive reactivity hyperaemia (PORH) testing on the same day. CMR imaging included T2 (oedema), native, and post-contrast T1 mapping to measure the extracellular volume fraction (ECV, fibrosis) and adenosine-stress-perfusion imaging measuring the relative myocardial upslope (microvascular coronary perfusion). Measures of peripheral microvascular function were related to CMR indices of oedema, fibrosis, and myocardial perfusion. SSc-PAH patients (n = 20) had higher T2 values and a trend towards a higher ECV, compared with IPAH patients (n = 5), and a lower nailfold capillary density (NCD) and reduced capillary recruitment after PORH. NCD correlated with ECV and T2 (r = -0.443 and -0.464, respectively, P < 0.05 for both) and with markers of diastolic dysfunction on echocardiography. PORH testing, but not NCD, correlated with the relative myocardial upslope (r = 0.421, P < 0.05)., Conclusion: SSc-PAH patients showed higher markers of cardiac fibrosis and inflammation, compared with IPAH patients. These markers correlated well with peripheral microvascular dysfunction, suggesting that SSc-driven inflammation and vasculopathy concurrently affect peripheral microcirculation and the heart. This may contribute to the disproportionate high mortality in SSc-PAH., Competing Interests: Conflict of interest: A.P.J.v.D. received a research grant from Janssen Pharmaceuticals and a speaker fee from Janssen. A.L.D. received a research grant from Actelion Pharmaceuticals and consulting fees from Janssen Pharmaceuticals and Merck. M.C.P. received research grants from the St. Antonius Hospital, Janssen Pharmaceuticals, and ZonMw. M.C.V. received research grants from Boehringer Ingelheim, Grupo Ferrer Internacional, Galapagos NV (Mechelen, Belgium) (Mechelen, Belgium), and Janssen Pharmaceuticals and consulting fees from Boehringer Ingelheim, Corbus Pharmaceuticals Holdings, and Janssen Pharmaceuticals. R.N. received research grants from Biotronik and Philips and consulting fees from Sanofi Genzyme, Bayer, and Bristol Myers Squib., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background: Pulmonary arterial hypertension is a serious medical condition. However, the condition is often misdiagnosed or a rather long delay occurs from symptom onset to diagnosis, associated with decreased 5-year survival. In this study, we developed and tested a deep-learning algorithm to detect pulmonary arterial hypertension using chest X-ray (CXR) images., Methods: From the image archive of Chiba University Hospital, 259 CXR images from 145 patients with pulmonary arterial hypertension and 260 CXR images from 260 control patients were identified; of which 418 were used for training and 101 were used for testing. Using the testing dataset for each image, the algorithm outputted a numerical value from 0 to 1 (the probability of the pulmonary arterial hypertension score). The training process employed a binary cross-entropy loss function with stochastic gradient descent optimization (learning rate parameter, α = 0.01). In addition, using the same testing dataset, the algorithm's ability to identify pulmonary arterial hypertension was compared with that of experienced doctors., Results: The area under the curve (AUC) of the receiver operating characteristic curve for the detection ability of the algorithm was 0.988. Using an AUC threshold of 0.69, the sensitivity and specificity of the algorithm were 0.933 and 0.982, respectively. The AUC of the algorithm's detection ability was superior to that of the doctors., Conclusion: The CXR image-derived deep-learning algorithm had superior pulmonary arterial hypertension detection capability compared with that of experienced doctors., (© 2024. The Author(s).)

Background: The 2022 European Society of Cardiology/European Respiratory Society pulmonary hypertension (PH) guidelines incorporate cardiac magnetic resonance (CMR) imaging metrics in the risk stratification of patients with pulmonary arterial hypertension (PAH). Thresholds to identify patients at estimated 1-year mortality risks of < 5%, 5% to 20%, and > 20% are introduced. However, these cutoff values are mostly single center-based and require external validation., Research Question: What are the discriminative prognostic properties of the current CMR risk thresholds stratifying patients with PAH?, Study Design and Methods: We analyzed data from incident, treatment-naïve patients with PAH from the Amsterdam University Medical Centres, Vrije Universiteit, The Netherlands. The discriminative properties of the proposed CMR three risk strata were tested at baseline and first reassessment, using the following PH guideline variables: right ventricular ejection fraction, indexed right ventricular end-systolic volume, and indexed left ventricular stroke volume., Results: A total of 258 patients with PAH diagnosed between 2001 and 2022 fulfilled the study criteria and were included in this study. Of these, 172 had follow-up CMR imaging after 3 months to 1.5 years. According to the CMR three risk strata, most patients were classified at intermediate risk (n = 115 [45%]) upon diagnosis. Only 29 (11%) of patients with PAH were classified at low risk, and 114 (44%) were classified at high risk. Poor survival discrimination was seen between risk groups. Appropriate survival discrimination was seen at first reassessment., Interpretation: Risk stratifying patients with PAH with the recent proposed CMR cutoffs from the European Society of Cardiology/European Respiratory Society 2022 PH guidelines requires adjustment because post-processing consensus is lacking and general applicability is limited. Risk assessment at follow-up yielded better survival discrimination, emphasizing the importance of the individual treatment response., Competing Interests: Financial/Nonfinancial Disclosures The authors have reported to CHEST the following: H. J. B. and A. V. N. were supported by research grants from Actelion, GSK, and Ferrer (Therabel). None declared (L. R. C., J. N. W., J. T. M., L. J. M., F. S. d. M.)., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Pulmonary arterial hypertension (PAH) is often a progressive, fatal disease. Because of nonspecificity of symptoms and limited awareness of PAH, patients are often diagnosed and referred late to accredited pulmonary hypertension (PH) centers, contributing to worsening survival and overall prognosis. The objective of the present study was to determine if the virtual echocardiography screening tool (VEST), a simple scoring system using routinely reported echocardiographic metrics, could capture earlier diagnoses of PAH before clinical recognition and referral to expert PH centers. This study is a retrospective analysis of 132 patients with PAH evaluated consecutively at 2 accredited referral PH centers. VEST scores and time to evaluation at PH center were quantified based on the first available echocardiogram before referral. Clinical risk assessment was calculated at initial evaluation by the PH center using the REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) 2.0 calculator. An overwhelming majority (93%) of the study participants had markedly abnormal VEST scores predictive of PAH before evaluation at a PH referral center. The median delay from VEST to evaluation was >6 months at 206 days (quartile 1, quartile 3: 55, 757). At initial evaluation, 72% were intermediate or high-risk based on REVEAL 2.0 risk assessment. In conclusion, we propose that VEST is a powerful yet simple scoring tool that can capture high-risk patients with PAH, prompting earlier diagnosis and referrals to accredited PH centers, and allowing for earlier expert implementation of PH medical therapies., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Cardiac magnetic resonance (CMR) is the gold standard technique to assess biventricular volumes and function, and is increasingly being considered as an end-point in clinical studies. Currently, with the exception of right ventricular (RV) stroke volume and RV end-diastolic volume, there is only limited data on minimally important differences (MIDs) reported for CMR metrics. Our study aimed to identify MIDs for CMR metrics based on US Food and Drug Administration recommendations for a clinical outcome measure that should reflect how a patient "feels, functions or survives"., Methods: Consecutive treatment-naïve patients with pulmonary arterial hypertension (PAH) between 2010 and 2022 who had two CMR scans (at baseline prior to treatment and 12 months following treatment) were identified from the ASPIRE registry. All patients were followed up for 1 additional year after the second scan. For both scans, cardiac measurements were obtained from a validated fully automated segmentation tool. The MID in CMR metrics was determined using two distribution-based (0.5sd and minimal detectable change) and two anchor-based (change difference and generalised linear model regression) methods benchmarked to how a patient "feels" (emPHasis-10 quality of life questionnaire), "functions" (incremental shuttle walk test) or "survives" for 1-year mortality to changes in CMR measurements., Results: 254 patients with PAH were included (mean±sd age 53±16 years, 79% female and 66% categorised as intermediate risk based on the 2022 European Society of Cardiology/European Respiratory Society risk score). We identified a 5% absolute increase in RV ejection fraction and a 17 mL decrease in RV end-diastolic or end-systolic volumes as the MIDs for improvement. Conversely, a 5% decrease in RV ejection fraction and a 10 mL increase in RV volumes were associated with worsening., Conclusions: This study establishes clinically relevant CMR MIDs for how a patient "feels, functions or survives" in response to PAH treatment. These findings provide further support for the use of CMR as a clinically relevant clinical outcome measure and will aid trial size calculations for studies using CMR., Competing Interests: Conflict of interest: A.J. Swift has received research grant funding from Janssen Pharmaceuticals, and consultancy fees from Janssen Pharmaceuticals and General Electric. D.G. Kiely has received grant funding from Ferrer and Janssen Pharmaceuticals, and speaker and consultancy fees and funding for travel from Acceleron, Altavant, Ferrer, Janssen, MSD and United Therapeutics. The other authors have no relationships relevant to the contents of this paper to disclose., (Copyright ©The authors 2023.)

Background: Current cardiac magnetic resonance (CMR) imaging in pulmonary arterial hypertension (PAH) focuses on measures of ventricular function and coupling., Objectives: The purpose of this study was to evaluate pulmonary artery (PA) global longitudinal strain (GLS) as a prognostic marker in patients with PAH., Methods: The authors included 169 patients with PAH from the ASPIRE (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre) and INITIATE (Integrated computatioNal modelIng of righT heart mechanIcs and blood flow dynAmics in congeniTal hEart disease) registries, and 82 normal controls with similar age and gender distributions. PA GLS was derived from CMR feature tracking. Right ventricular measurements including volumes, ejection fraction, and right ventricular GLS were also derived from CMR. Patients were followed up a median of 34 months with all-cause mortality as the primary endpoint. Other known risk scores were collected, including the REVEAL (Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) 2.0 and COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) 2.0 scores., Results: Of 169 patients (mean age: 57 ± 15 years; 80% female), 45 (26.6%) died (median follow-up: 34 months). Mean PA GLS was 23% ± 6% in normal controls and 10% ± 5% in patients with PAH (P < 0.0001). Patients with PA GLS <9% had a higher risk of mortality than those with PA GLS ≥9% (P < 0.001), and this was an independent predictor of mortality in PAH on multivariable analysis after adjustment for known risk factors (HR: 2.93; P = 0.010). Finally, in patients with PAH, PA GLS provided incremental prognostic value over the REVEAL 2.0 (global chi-square; P = 0.001; C statistic comparison; P = 0.030) and COMPERA 2.0 (global chi-square; P = 0.001; C statistic comparison; P = 0.048)., Conclusions: PA GLS confers incremental prognostic utility over the established risk scores for identifying patients with PAH at higher risk of death, who may be targeted for closer monitoring and/or intensified therapy., Competing Interests: Funding Support and Author Disclosures This study has received funding support from the National Medical Research Council of Singapore (NMRC/OFIRG/0018/2016). Dr Kiely has received personal fees for consultancy work, educational talks and participation in steering committees from Acceleron, Ferrer, GSK, MSD, and Janssen; and has received funding from the National Institute for Health and Care Research, Sheffield Biomedical Research Centre. The funder had no role in the design and conduct of the study; collection; management, analysis, and interpretation of the data; and preparation, review, or approval of the manuscript. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Magnetic resonance imaging - is high precision method for diagnosing cardiovascular diseases. Simultaneously with the anatomy and function of the right ventricle, magnetic resonance imaging allows to assess the pulmonary circulation, which leads to the widespread use of this method in the diagnosis and dynamic monitoring of patients with pulmonary arterial hypertension. The article is devoted to the assessment of cardiac remodeling and risk stratification of this group of patients. Special attention is given to new prognostic parameters included in the scale for risk stratification of patients with pulmonary hypertension of the European Society of Cardiology/European Respiratory Society 2022.

Background: Multiparametric risk assessment tools determine mortality risk in patients with pulmonary arterial hypertension (PAH) by combining invasive and noninvasive variables so management strategies can be tailored to individuals., Research Question: Can a risk score based on common echocardiographic parameters risk-stratify patients with PAH?, Study Design and Methods: A Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) echocardiographic risk score (REVEAL-ECHO) was derived using retrospective echocardiographic data from 2,400 adult patients with PAH enrolled in the REVEAL registry database. A stepwise Cox regression model identified echocardiographic parameters significantly predictive of survival. Values were assigned to each selected parameter based on survival at 12 months' follow-up (Kaplan-Meier estimates). The REVEAL-ECHO risk score was the sum of individual values. Patients were categorized as having low, intermediate, or high risk based on Kaplan-Meier-predicted 12-month survival., Results: The risk score included four echocardiographic parameters-right ventricular (RV) chamber enlargement, reduced RV systolic function, tricuspid regurgitation severity, and pericardial effusion-and accounted for PAH etiology. Higher REVEAL-ECHO risk scores signaled lower probability of 12-month survival. Statistically significant separation of mortality risk was observed among the risk strata: intermediate vs low (hazard ratio [HR], 1.43; 95% CI, 1.17-1.75; P = .0004) and high vs low (HR, 2.60; 95% CI, 2.19-3.10; P < .0001). Augmentation of the REVEAL Lite 2 risk calculator with REVEAL-ECHO risk scores achieved separation of REVEAL Lite 2 into four risk groups and identified a subgroup of patients with a low REVEAL Lite 2 risk score who were at higher risk (intermediate-low risk) and a subgroup of patients with an intermediate REVEAL Lite 2 risk score who also were at higher risk (intermediate-high risk)., Interpretation: A REVEAL-ECHO risk score, derived using four echocardiographic parameters, may discriminate risk further when used as an adjunct to current risk assessment scores. Further validation is required., (Copyright © 2023. Published by Elsevier Inc.)

Background: Cardiovascular magnetic resonance (CMR) offers comprehensive right ventricular (RV) evaluation in pulmonary arterial hypertension (PAH). Emerging four-dimensional (4D) flow CMR allows visualization and quantification of intracardiac flow components and calculation of phasic blood kinetic energy (KE) parameters but it is unknown whether these parameters are associated with cardiopulmonary exercise test (CPET)-assessed exercise capacity, which is a surrogate measure of survival in PAH. We compared 4D flow CMR parameters in PAH with healthy controls, and investigated the association of these parameters with RV remodelling, RV functional and CPET outcomes., Methods: PAH patients and healthy controls from two centers were prospectively enrolled to undergo on-site cine and 4D flow CMR, and CPET within one week. RV remodelling index was calculated as the ratio of RV to left ventricular (LV) end-diastolic volumes (EDV). Phasic (peak systolic, average systolic, and peak E-wave) LV and RV blood flow KE indexed to EDV (KEI EDV ) and ventricular LV and RV flow components (direct flow, retained inflow, delayed ejection flow, and residual volume) were calculated. Oxygen uptake (VO 2 ), carbon dioxide production (VCO 2 ) and minute ventilation (VE) were measured and recorded., Results: 45 PAH patients (46 ± 11 years; 7 M) and 51 healthy subjects (46 ± 14 years; 17 M) with no significant differences in age and gender were analyzed. Compared with healthy controls, PAH had significantly lower median RV direct flow, RV delayed ejection flow, RV peak E-wave KEI EDV , peak VO 2 , and percentage (%) predicted peak VO 2 , while significantly higher median RV residual volume and VE/VCO 2 slope. RV direct flow and RV residual volume were significantly associated with RV remodelling, function, peak VO 2 , % predicted peak VO 2 and VE/VCO 2 slope (all P < 0.01). Multiple linear regression analyses showed RV direct flow to be an independent marker of RV function, remodelling and exercise capacity., Conclusion: In this 4D flow CMR and CPET study, RV direct flow provided incremental value over RVEF for discriminating adverse RV remodelling, impaired exercise capacity, and PAH with intermediate and high risk based on risk score. These data suggest that CMR with 4D flow CMR can provide comprehensive assessment of PAH severity, and may be used to monitor disease progression and therapeutic response., Trial Registration Number: https://www., Clinicaltrials: gov . Unique identifier: NCT03217240., (© 2022. The Author(s).)

Background Right heart failure may lead to impaired liver perfusion and venous congestion, resulting in different extents of liver fibrosis. However, whether hepatic tissue deterioration determined by native T1 mapping and extracellular volume fraction using cardiac magnetic resonance imaging is associated with poor outcomes in patients with pulmonary arterial hypertension remains unclear. Methods and Results A total of 131 participants with pulmonary arterial hypertension (mean age, 36±13 years) and 64 healthy controls (mean age, 44±18) between October 2013 and December 2019 were prospectively enrolled. Hepatic native T1 and extracellular volume fraction values were measured using modified Look-Locker inversion recovery T1 mapping sequences. The primary end point was all-cause mortality; the secondary end point was all-cause mortality and repeat hospitalization attributable to heart failure. Cox regression models and Kaplan-Meier survival analysis were used to identify the association between variables and clinical outcome. During a median follow-up of 34.5 months (interquartile range: 25.3-50.8), hepatic native T1 (hazard ratio per 30-ms increase, 1.22 [95% CI, 1.07-1.39]; P =0.003) and extracellular volume fraction (hazard ratio per 3% increase, 1.18 [95% CI, 1.04-1.34]; P =0.010) values were associated with a higher risk of death. In the multivariate Cox model, hepatic native T1 value (hazard ratio per 30-ms increase, 1.15 [95% CI, 1.04-1.27]; P =0.009) remained as an independent prognostic factor for the secondary end point. Conclusions Hepatic T1 mapping values were predictors of adverse cardiovascular events in participants with pulmonary arterial hypertension and could be novel imaging biomarkers for poor prognosis recognition.

We aimed to research the role of right ventricular strain parameters (RVSP) quantified by cardiac magnetic resonance feature tracking (CMR-FT) in the early assessment of right ventricular (RV) function in patients with pulmonary arterial hypertension associated with atrial septal defect (PAH-ASD). From September 2017 to May 2021, we retrospectively enrolled 41 patients with PAH-ASD and 20 healthy controls. All subjects underwent CMR-FT, and right heart catheterization was conducted in patients with PAH-ASD. The relationship between RVSP and RV functional parameters was subjected to correlation analysis, and intragroup correlation coefficient (ICC) and Bland-Altman plots were used to assess the consistency. The subjects were divided into three groups: Group A (controls; n = 20), Group B (PAH-ASD, RVEF ≥ 45%; n = 14), and Group C (PAH-ASD, RVEF < 45%; n = 27). Compared with healthy controls, the RV global longitudinal strain (GLS) in Group B was significantly decreased (- 19.68 ± 2.72% vs. - 25.21 ± 3.6%, P < 0.05). In RVEF-preserved PAH-ASD patients (Group B), compared with patients with GLS ≤ - 20%, patients with GLS > - 20% also had significantly elevated right ventricular end-diastolic pressure (RVEDP) [8 (6.5-8.25) mmHg vs. 4.5 ± 1.64 mmHg, P < 0.05]. RV GLS had a moderate to strong correlation with RVEF, RVESVi, RVEDVi, RVEDP, and NT-proBNP (P < 0.05). ICC and Bland-Altman plots showed good intragroup and intergroup consistency in radial, circumferential and longitudinal strains of RV. In conclusion, it is feasible to quantify RV strain in patients with PAH-ASD by CMR-FT, and GLS is valuable for the early assessment of RV dysfunction in patients with PAH-ASD., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Objectives: To determine the prognostic value of patterns of right ventricular adaptation in patients with pulmonary arterial hypertension (PAH), assessed using cardiac magnetic resonance (CMR) imaging at baseline and follow-up., Methods: Patients attending the Sheffield Pulmonary Vascular Disease Unit with suspected pulmonary hypertension were recruited into the ASPIRE (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral Centre) Registry. With exclusion of congenital heart disease, consecutive patients with PAH were followed up until the date of census or death. Right ventricular end-systolic volume index adjusted for age and sex and ventricular mass index were used to categorise patients into four different volume/mass groups: low-volume-low-mass, low-volume-high-mass, high-volume-low-mass and high-volume-high-mass. The prognostic value of the groups was assessed with one-way analysis of variance and Kaplan-Meier plots. Transition of the groups was studied., Results: A total of 505 patients with PAH were identified, 239 (47.3%) of whom have died at follow-up (median 4.85 years, IQR 4.05). The mean age of the patients was 59±16 and 161 (32.7%) were male. Low-volume-low-mass was associated with CMR and right heart catheterisation metrics predictive of improved prognosis. There were 124 patients who underwent follow-up CMR (median 1.11 years, IQR 0.78). At both baseline and follow-up, the high-volume-low-mass group had worse prognosis than the low-volume-low-mass group (p<0.001). With PAH therapy, 73.5% of low-volume-low-mass patients remained in this group, whereas only 17.4% of high-volume-low-mass patients transitioned into low-volume-low-mass., Conclusions: Right ventricular adaptation assessed using CMR has prognostic value in patients with PAH. Patients with maladaptive remodelling (high-volume-low-mass) are at high risk of treatment failure., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY. Published by BMJ.)

Background: Systemic lupus erythematosus (SLE) accounts for the largest portion of connective tissue disease-associated pulmonary arterial hypertension (PAH) in Asian countries, especially in China, and SLE-PAH poses multiple challenges during pregnancy and delivery. Patients with SLE-PAH tend to have lower survival rates and worse quality of life than other subgroups of PAH., Case Presentation: Presented in this report is a 28-year-old primipara who suffered from SLE for 13 years and SLE-PAH for nine years. She had cardiac care throughout these years. She was admitted at 26 weeks of gestation for progressive dyspnea on exertion and her condition improved after a three-week PAH-targeted therapy consisting of prostacyclin and PDE-5 inhibitor. At 29 weeks of gestation, she was infected with influenza H1N1 and her clinical status deteriorated with increased dyspnea. After two weeks of influenza therapy and maximization of PAH therapy, a cesarean delivery was performed under epidural anesthesia at 31 weeks of gestation. She was discharged ten days after delivery. Although the targeted therapy for both PAH and SLE was readjusted after delivery and regular follow-up showed a gradual recovery and a stable condition, she still died suddenly at home 12 months after delivery. The child is healthy., Conclusions: Sequential combination therapy of PAH and SLE and the structured perinatal management might lead to optimal short-term outcomes in the mother and fetus. Long-term outcomes in women with PAH who become pregnant are poor, with high rates of morbidity and mortality. Delivery strategies remain an important challenge for modern Pregnancy Heart Teams., (Copyright © 2021. Published by Elsevier Inc.)

Objective: Early detection of pulmonary arterial hypertension (PAH) is crucial for improving patient outcomes. The aim of this study was to compare the positive predictive value (PPV) of the echocardiography-derived tricuspid annular plane systolic excursion/systolic pulmonary artery pressure (TAPSE/sPAP) ratio with that of the DETECT algorithm for PAH screening in a cohort of SSc patients., Methods: Fifty-one SSc patients were screened for PAH using the DETECT algorithm and echocardiography., Results: Echocardiography was recommended by the DETECT algorithm step 1 in 34 patients (66.7%). Right heart catheterization (RHC) was recommended by the DETECT algorithm step 2 in 16 patients (31.4%). PAH was confirmed by RHC in 5 patients. The DETECT algorithm PPV was 31.3%. The TAPSE/sPAP ratio was higher in SSc patients not referred for RHC than in SSc patients referred for RHC according to the DETECT algorithm step 2 [0.83 (0.35-1.40) mm/mmHg vs 0.74 (0.12-1.09)  mm/mmHg, P < 0.05]. Using a cut-off of 0.60 mm/mmHg, 8 (15.7%) SSc patients had a TAPSE/sPAP ratio of ≤0.60 mm/mmHg. PAH was confirmed by RHC in 5 patients. The PPV of TAPSE/sPAP was 62.5%. In multiple regression analysis, TAPSE/sPAP was associated with age [β coefficient = -0.348 (95% CI: -0.011, -0.003); P < 0.01], DETECT algorithm step 1 [β coefficient = 1.023 (95% CI: 0.006, 0.024); P < 0.01] and DETECT algorithm step 2 (β coefficient = -1.758 [95% CI: -0.059, -0.021]; P < 0.0001)., Conclusion: In SSc patients with a DETECT algorithm step 2 total score of >35, the TAPSE/sPAP ratio can be used to further select patients requiring RHC to confirm PAH diagnosis., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Background: Risk stratification is central to the management of pulmonary arterial hypertension (PAH). For this purpose, multiparametric tools have been developed, including the ESC/ERS risk score and its simplified versions derived from large database analysis such as the COMPERA and the French Pulmonary Hypertension Network (FPHN) registries. However, the distinction between high and intermediate-risk profiles may be difficult as the latter lacks granularity. In addition, neither COMPERA or FPHN strategies included imaging-derived markers. We thus aimed at investigating whether surrogate echocardiographic markers of right ventricular (RV) to pulmonary artery (PA) coupling could improve risk stratification in patients at intermediate-risk., Material and Methods: A single-center retrospective analysis including 102 patients with a diagnosis of PAH was performed. COMPERA and FPHN strategies were applied to stratify clinical risk. The univariate linear regression was used to test the influence of the echo-derived parameters qualifying the right heart (right ventricle basal diameter, right atrial area, and pressure, tricuspid regurgitation velocity, tricuspid annular plane systolic excursion -TAPSE-). Among these, the TAPSE and tricuspid regurgitation velocity ratio (TAPSE/TRV) as well as the TAPSE and systolic pulmonary artery pressure ratio (TAPSE/sPAP) were considered as surrogate of RV-PA coupling., Results: TAPSE/TRV and TAPSE/sPAP resulted the more powerful markers of prognosis. Once added to COMPERA, TAPSE/TRV or TAPSE/sPAP significantly dichotomized intermediate-risk group in intermediate-to-low-risk (TAPSE/TRV≥3.74 mm∙nm/s)-1 or TAPSE/sPAP≥0.24 mm/mmHg) and in intermediate-to-high-risk subgroups (TAPSE/TRV<3.74 mm∙(m/s)-1 or TAPSE/sPAP<0.24 mm/mmHg). In the same way, TAPSE/TRV or TAPSE/sPAP was able to select patients at lower risk among those with 2, 1, and 0 low-risk criteria of both invasive and non-invasive FPHN registries., Conclusions: Our results suggest that adopting functional-hemodynamic echo-derived parameters may provide a more accurate risk stratification in patients with PAH. In particular, TAPSE/TRV or TAPSE/sPAP improved risk stratification in patients at intermediate-risk, that otherwise would have remained less characterized., Competing Interests: The authors have declared that no competing interests exist.

Background: Pulmonary arterial hypertension (PAH) is a complex and severe complication of connective tissue disease (CTD). We aimed to evaluate the application value of myocardial perfusion imaging (MPI) in evaluating CTD-associated PAH (CTD-PAH)., Methods: We retrospectively included 88 patients who were diagnosed with CTD between January 2018 and December 2020 at our hospital. Fifty-eight patients had PAH and were included into the CTD-PAH group. Thirty patients without PAH were included in the control group. All patients received routine physical examination, biochemical tests and cardiac function evaluation, right heart catheterization (RHC), and 99m Tc-MIBI MPI. PAH patients were divided into the mild, moderate, and severe PAH group according to their mean pulmonary artery pressures by RHC. Pearson correlation analysis was used to calculate the correlation between the right ventricle target/background (T/B) and right ventricle stroke volume (RV-SV), total pulmonary resistance (TPR), pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), 6-minute walk distance (6-MWD), and N-terminal B-type natriuretic peptide (NT-proBNP). The ROC curves of T/B and pulmonary artery pressure classification were plotted and the sensitivity and specificity of T/B in diagnosing PAH of different severities were analyzed., Results: The analysis of correlation revealed that T/B correlated negatively with 6-MWD and positively with NT-proBNP and exhibited good positive correlation with mPAP, TPR, and PVR by RHC and negative correlation with RV-SV. T/B was of the most diagnostic value for severe PAH, and its correlation with severe PAH was stronger than that with mild PAH and moderate PAH., Conclusions: Target/background is a noninvasive method that can simultaneously evaluate pulmonary arterial pressure and myocardial perfusion of CTD-CHD patients and is particularly of relatively high value for severe PAH patients., (© 2021 The Authors. Annals of Noninvasive Electrocardiology published by Wiley Periodicals LLC.)

Pulmonary arterial hypertension (PAH) patients eventually die of right heart failure (RHF). Currently, there is no suitable pre-clinical model to study PAH. Therefore, we aim to develop a right heart dysfunction (RHD) model using the 3-dimensional engineered heart tissue (EHT) approach and cardiomyocytes derived from patient-induced pluripotent stem cells (iPSCs) to unravel the mechanisms that determine the fate of a pressure-overloaded right ventricle. iPSCs from PAH and healthy control subjects were differentiated into cardiomyocytes (iPSC-CMs), incorporated into the EHT, and maintained for 28 days. In comparison with control iPSC-CMs, PAH-derived iPSC-CMs exhibited decreased beating frequency and increased contraction and relaxation times. iPSC-CM alignment within the EHT was observed. PAH-derived EHTs exhibited higher force, and contraction and relaxation times compared with control EHTs. Increased afterload was induced using 2× stiffer posts from day 0. Due to high variability, there were no functional differences between normal and stiffer EHTs, and no differences in the hypertrophic gene expression. In conclusion, under baseline spontaneous conditions, PAH-derived iPSC-CMs and EHTs show prolonged contraction compared with controls, as observed clinically in PAH patients. Further optimization of the hypertrophic model and profound characterization may provide a platform for disease modelling and drug screening.

Background: Pulmonary hypertension is a heterogeneous disease, and a significant portion of patients at risk for it have CT imaging available. Advanced automated processing techniques could be leveraged for early detection, screening, and development of quantitative phenotypes. Pruning and vascular tortuosity have been previously described in pulmonary arterial hypertension (PAH), but the extent of these phenomena in arterial vs venous pulmonary vasculature and in exercise pulmonary hypertension (ePH) have not been described., Research Question: What are the arterial and venous manifestations of pruning and vascular tortuosity using CT imaging in PAH, and do they also occur in ePH?, Study Design and Methods: A cohort of patients with PAH and ePH and control subjects with available CT angiograms were retrospectively identified to examine the differential arterial and venous presence of pruning and tortuosity in patients with precapillary pulmonary hypertension not confounded by lung or thromboembolic disease. The pulmonary vasculature was reconstructed, and an artificial intelligence method was used to separate arteries and veins and to compute arterial and venous vascular volumes and tortuosity., Results: A total of 42 patients with PAH, 12 patients with ePH, and 37 control subjects were identified. There was relatively lower (median [interquartile range]) arterial small vessel volume in subjects with PAH (PAH 14.7 [11.7-16.5; P < .0001]) vs control subjects (16.9 [15.6-19.2]) and venous small vessel volume in subjects with PAH and ePH (PAH 8.0 [6.5-9.6; P < .0001]; ePH, 7.8 [7.5-11.4; P = .004]) vs control subjects (11.5 [10.6-12.2]). Higher large arterial volume, however, was only observed in the pulmonary arteries (PAH 17.1 [13.6-23.4; P < .0001] vs control subjects 11.4 [8.1-15.4]). Similarly, tortuosity was higher in the pulmonary arteries in the PAH group (PAH 3.5 [3.3-3.6; P = .0002] vs control 3.2 [3.2-3.3])., Interpretation: Lower small distal pulmonary vascular volume, higher proximal arterial volume, and higher arterial tortuosity were observed in PAH. These can be quantified by using automated techniques from clinically acquired CT scans of patients with ePH and resting PAH., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Right ventricular function is an important prognostic marker for pulmonary arterial hypertension. Native T1 mapping using cardiovascular magnetic resonance imaging can characterize the myocardium, but accumulating evidence indicates that T1 values of the septum or ventricular insertion points do not have predictive potential in pulmonary arterial hypertension. We aimed to elucidate whether native T1 values of the right ventricular free wall (RVT1) can predict poor outcomes in patients with pulmonary arterial hypertension., Methods: This retrospective study included 30 patients with pulmonary arterial hypertension (median age, 45 years; mean pulmonary artery pressure, 41±13 mmHg) and 16 healthy controls (median age, 43 years) who underwent native T1 mapping. RVT1 was obtained from the inferior right ventricular free wall during end systole., Results: Patients with pulmonary arterial hypertension had significantly higher native RVT1 than did controls (1384±74 vs. 1217±57 ms, p<0.001). Compared with T1 values of the septum or ventricular insertion points, RVT1 correlated better with the effective right ventricular elastance index (R = -0.53, p = 0.003), ventricular-arterial uncoupling (R = 0.46, p = 0.013), and serum brain natriuretic peptide levels (R = 0.65, p<0.001). Moreover, the baseline RVT1 was an accurate predictor of the reduced right ventricular ejection fraction at the 12-month follow-up (delta -3%). RVT1 was independently associated with composite events of death or hospitalization from any cause (hazard ratio = 1.02, p = 0.002)., Conclusions: RVT1 was predictive of right ventricular performance and outcomes in patients with pulmonary arterial hypertension. Thus, native T1 mapping in the right ventricular free wall may be an effective prognostic method for pulmonary arterial hypertension., Competing Interests: We have read the journal’s policy and the authors of this manuscript have the following competing interests: Ryotaro Asano, Takeshi Ogo, and Mark T. Waddingham belong to a department endowed by Nippon Shinyaku Co. The remaining authors have nothing to disclose regarding the publication of this article. This does not alter our adherence to PLOS ONE policies on sharing data and materials.

In addition to being an antioxidant, thioredoxin (Trx) is known to stimulate signaling pathways involved in cell proliferation and to inhibit apoptosis. The aim of this study was to explore the role of Trx in some of these pathways along the progression of monocrotaline (MCT)-induced pulmonary arterial hypertension (PAH). Male rats were first divided into two groups: monocrotaline (MCT - 60 mg/kg i.p.) and control (received saline), that were further divided into three groups: 1, 2, and 3 weeks. Animals were submitted to echocardiographic analysis. Right and left ventricles were used for the measurement of hypertrophy, through morphometric and histological analysis. The lung was prepared for biochemical and molecular analysis. One week after MCT injection, there was an increase in thioredoxin reductase (TrxR) activity, a reduction in glutathione reductase (GR) activity, and an increase in Trx-1 and vitamin D3 up-regulated protein-1 (VDUP-1) expression. Two weeks after MCT injection, there was an increase in VDUP-1, Akt and cleaved caspase-3 activation, and a decrease in Trx-1 and Nrf2 expression. PAH-induced by MCT promoted a reduction in Nrf2 and Trx-1 expression as well as an increase in Akt and VDUP-1 expression after three weeks. The increase in pulmonary vascular resistance was accompanied by increased TrxR activity, suggesting an association between the Trx system and functional changes in the progression of PAH. It seems that Trx-1 activation was an adaptive response to MCT administration to cope with pulmonary remodeling and disease progression, suggesting a potential new target for PAH therapeutics., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Metabolism is essential for a living organism to sustain life. It provides energy to a cell by breaking down compounds (catabolism) and supplies building blocks for the synthesis of macromolecules (anabolism). Signal transduction pathways tightly regulate mammalian cellular metabolism. Simultaneously, metabolism itself serves as a signaling pathway to control many cellular processes, such as proliferation, differentiation, cell death, gene expression, and adaptation to stress. Considerable progress in the metabolism field has come from understanding how cancer cells co-opt metabolic pathways for growth and survival. Recent data also show that several metabolic pathways may participate in the pathogenesis of lung diseases, some of which could be promising therapeutic targets. In this translational review, we will outline the basic metabolic principles learned from the cancer metabolism field as they apply to the pathogenesis of pulmonary arterial hypertension and fibrosis and will place an emphasis on therapeutic potential.

Pulmonary arterial hypertension (PAH) represents one of the most devastating complications in connective tissue diseases (CTDs). The aim of this study was to investigate the presence of peripheral microangiopathy in patients with PAH associated with CTDs (CTD-PAH) by exploring nailfold videocapillaroscopic (NVC) changes and identify possible associations of NVC characteristics with markers of disease severity. Α cross-sectional study was performed in 18 CTD-PAH patients [13 PAH due to systemic sclerosis (SSc-PAH) and 5 with other types of CTD-PAH], 14 patients with SSc without PAH (SSc-non-PAH) and 20 healthy controls. NVC quantitative and qualitative parameters were evaluated using Optilia Digital Capillaroscope. To ensure inter-observer repeatability, capillaroscopic images were reviewed by two independent investigators. When compared to healthy controls, patients with CTD-PAH (77.8% women, mean age 65.9 years) presented reduced capillary density (6.5 ± 1.6 loops/mm vs. 9.7 ± 0.7 loops/mm, p < 0.001) and increased capillary loop width (23.3 ± 10.1 μm vs. 11.2 ± 2.5 μm, p < 0.001). SSc-PAH patients presented lower capillary density in comparison with other CTD-PAH patients and SSc-non-PAH subjects and abnormal and disorganized capillaries compared to controls. Patients with other CTD-PAH had also reduced capillary density and increased loop diameter compared to controls. A significant linear correlation was identified between capillary density and estimated glomerular filtration rate in the total CTD-PAH population (r = 0.63, p = 0.007). In SSc-PAH group, capillary loop diameter was positively correlated to cardiac index (r = 0.61, p = 0.02). Significant NVC microvascular changes were detected in patients with various types of CTD-PAH, suggesting an impaired peripheral microcirculation parallel to pulmonary vasculopathy.

Aims: We examined the effects of treatment with 1-nitro-2-phenylethane (NP), a novel soluble guanylate cyclase stimulator, on monocrotaline (MCT)-induced PAH in rats., Main Methods: At day 0, male adult rats were injected with a single subcutaneous (s.c.) dose of monocrotaline (60 mg/kg). Control (CNT) rats received an equal volume of monocrotaline's vehicle only (s.c.). Four weeks later, MCT-treated rats were treated orally for 14 days with NP (50 mg/kg/day) (MCT-NP group) or its vehicle (Tween 2%) (MCT-V group). At the end of the treatment period and before invasive hemodynamic study, rats of all experimental groups were examined by echocardiography., Key Findings: With respect to CNT rats, MCT-V rats showed significant; (1) increases in pulmonary artery (PA) diameter, RV free wall thickness and end-diastolic RV area, and increase of Fulton index; (2) decreases in maximum pulmonary flow velocity, PA acceleration time (PAAT), PAAT/time of ejection ratio, and velocity-time integral; (3) increases in estimated mean pulmonary arterial pressure; (4) reduction of maximal relaxation to acetylcholine in aortic rings, and (5) increases in wall thickness of pulmonary arterioles. All these measured parameters were significantly reduced or even abolished by oral treatment with NP., Significance: NP reversed endothelial dysfunction and pulmonary vascular remodeling, which in turn reduced ventricular hypertrophy. NP reduced pulmonary artery stiffness, normalized the pulmonary artery diameter and alleviated RV enlargement. Thus, NP may represent a new therapeutic or a complementary approach to treatment of PAH., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background and Purpose: Patients with pulmonary arterial hypertension (PAH) frequently present with anxiety, depression, autonomic, and cognitive deterioration, which may indicate brain changes in regions that control these functions. However, the precise regional brain-injury in sites that regulate cognitive, autonomic, and mood functions in PAH remains unclear. We examined the shifts in regional gray matter (GM) volume, using high-resolution T1-weighted images, and brain tissue alterations, using T2-relaxometry procedures, in PAH compared to healthy subjects., Methods: We collected two high-resolution T1-weighted series, and proton-density and T2-weighted images using a 3.0-Tesla magnetic resonance imaging scanner from 9 PAH and 19 healthy subjects. Both high-resolution T1-weighted images were realigned and averaged, partitioned to GM tissue type, normalized to a common space, and smoothed. Using proton-density and T2-weighted images, T2-relaxation maps were calculated, normalized to a common space, and smoothed. Whole-brain GM volume and T2-relaxation maps were compared between PAH and controls using analysis of covariance (covariates, age, sex, and total-brain-volume; false discover rate corrections)., Results: Significantly decreased GM volumes, indicating tissue injury, emerged in multiple brain regions, including the hippocampus, insula, cerebellum, parahippocampus, temporal, frontal, and occipital gyri, cingulate, amygdala, and thalamus. Higher T2-relaxation values, suggesting tissue damage, appeared in the cerebellum, hippocampus, parahippocampus, frontal, lingual, and temporal and occipital gyri, and cingulate areas in PAH compared to healthy subjects., Conclusions: PAH patients showed significant GM injury and brain tissue changes in sites that regulate cognition, autonomic, and mood functions. These findings indicate a brain structural basis for functional deficits in PAH patients., (© 2021 American Society of Neuroimaging.)

Background: Parameters of myocardial deformation may provide improved insights into right ventricular (RV) dysfunction. We quantified RV longitudinal myocardial function using a fast, semi-automated method and investigated its diagnostic and prognostic values in patients with repaired tetralogy of Fallot (rTOF) and pulmonary arterial hypertension (PAH), who respectively exemplify patients with RV volume and pressure overload conditions., Methods: The study enrolled 150 patients (rTOF, n = 75; PAH, n = 75) and 75 healthy controls. RV parameters of interest were fast global longitudinal strain (GLS) and strain rates during systole (GLSR s ), early diastole (GLSR e ) and late diastole (GLSR a ), obtained by tracking the distance from the medial and lateral tricuspid valve insertions to the RV epicardial apex on cine cardiovascular magnetic resonance (CMR)., Results: The RV fast GLS exhibited good agreement with strain values obtained by conventional feature tracking approach (bias - 4.9%, error limits (± 2·standard deviation) ± 4.3%) with fast GLS achieving greater reproducibility and requiring reduced analysis time. Mean RV fast GLS was reduced in PAH and rTOF groups compared to healthy controls (PAH < rTOF < healthy controls: 15.1 ± 4.9 < 19.3 ± 2.4 < 24.4 ± 3.0%, all P < 0.001 in pairwise comparisons). In rTOF patients, RV fast GLS was significantly associated with metabolic equivalents, peak oxygen consumption (PVO 2 ) and percentage of predicted PVO 2 achieved during cardiopulmonary exercise testing. Lower RV fast GLS was associated with subnormal exercise capacity in rTOF (area under the curve (AUC) = 0.822, sensitivity = 72%, specificity = 91%, cut-off = 19.3%). In PAH patients, reduced RV fast GLS was associated with RV decompensated hemodynamics (AUC = 0.717, sensitivity = 75%, specificity = 58%, cut-off = 14.6%) and higher risk of clinical worsening (AUC = 0.808, sensitivity = 79%, specificity = 70 %, cut-off = 16.0%)., Conclusions: Quantitative RV fast strain and strain rate parameters assessed from CMR identify abnormalities of RV function in rTOF and PAH and are predictive of exercise capacity, RV decompensation and clinical risks in these patients. Trial registry Clinicaltrials.gov: NCT03217240.

Background: Pulmonary arterial hypertension (PAH) is characterized by the right ventricle (RV) remodeling and pulmonary endothelial dysfunction. We studied cardiac perfusion and innervation in PAH with a cadmium-zinc-telluride (CZT) scanner and lung uptake impairment of 123 I-metaiodobenzylguanidine ( 123 I-MIBG)., Methods: In 13 patients with newly diagnosed PAH and 11 dilated cardiomyopathies (DCM, for comparative purposes), we assessed early and delayed 123 I-MIBG uptake ratios of lung-to-mediastinum (L/M) and heart-to-mediastinum (H/M) on anterior planar images. A quantitative myocardial innervation with 123 I-MIBG and perfusion with 99m Tc-tetrofosmin using CZT-SPECT was performed. All patients underwent right heart catheterization., Results: Early and delayed L/M ratios in PAH were lower than DCM (1.47 ± 0.14 vs 1.98 ± 0.11 and 1.40 ± 0.13 vs 1.83 ± 0.09; P < .001), while early and delayed H/M were impaired but not different (1.73 ± 0.20 vs 1.65 ± 0.18 and 1.73 ± 0.27 vs 1.58 ± 0.19). RV perfusion and early innervation were significantly higher in PAH compared to DCM (68.4 ± 13.4 vs 28.6 ± 4.1 and 58.8 ± 9.3 vs 27 ± 2.2; P < .001); delayed RV innervation was not evaluable. RV/LV perfusion and innervation ratios were significantly related (R = 0.74; P < .0001) and had a significant correlation with clinical, hemodynamic, and morpho-functional parameters, including L/M ratios., Conclusion: Cardio-pulmonary scintigraphy through a perfusion and innervation study is feasible and may identify pulmonary vascular and RV remodeling, as in PAH.

Objectives: Despite advances in treatment of idiopathic pulmonary arterial hypertension (IPAH), there remains no medical cure, and patients can experience disease progression leading to right heart failure, progressive exercise intolerance, and death. The reversed Potts shunt (left pulmonary artery to descending aorta) was reintroduced for treatment of end-stage IPAH to permit decompression of the suprasystemic right ventricle by right to left shunting, with preservation of upper body oxygenation. The shunt has the potential to delay the need for lung transplantation and offer a treatment for those who are transplant ineligible. To optimize shunt design and avoid the potential complications of bidirectional shunting, we developed a novel approach using a unidirectional-valved shunt (UVS) in patients with IPAH with suprasystemic pulmonary arterial pressure and poor right ventricular function., Methods: A single-center retrospective review was performed of UVS cases done at Columbia University Medical Center-New York Presbyterian between November 1, 2016, and May 1, 2019., Results: Five patients (4 female; ages 12-22 years) underwent UVS. All had suprasystemic pulmonary arterial pressure, poor right ventricular function, and World Health Organization functional class IV symptoms at baseline. All patients are alive and transplant-free at latest follow-up (range 3-33 months; median 6 ± 11 months)., Conclusions: The UVS may offer an alternative solution to lung transplantation in adolescents and young adults with IPAH. Longer-term follow-up is needed to determine the ultimate impact of unidirectional unloading of the right ventricle in these patients and to determine whether the UVS will enable a broader approach to the treatment of patients with IPAH., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Pulmonary hypertension (PH) is an underdiagnosed and potentially fatal condition. The utility of screening for PH in hospitalized patients undergoing echocardiography is unknown. The goal of this study was to determine the prevalence of undiagnosed pulmonary hypertension (PH) and probable pulmonary arterial hypertension (PAH) in hospitalized patients undergoing echocardiography for any indication. All hospitalized patients undergoing echocardiography were identified and echocardiographs reviewed prospectively for the presence of a tricuspid regurgitant (TR) jet. Electronic medical records (EMR) of patients with a TR jet ≥ 3 m/s were reviewed for identifiable causes of pulmonary hypertension. Patients with no identifiable cause were classified as presumptive World Health Organization (WHO) Group 1 PH (also known as PAH). These PAH patients were compared to other PH patients for baseline demographic characteristics and comorbidities as well as 30-day readmission and mortality. The admitting physicians of patients classified as PH were advised to consider further evaluation including right heart catheterization. We reviewed 4417 consecutive echocardiograms and identified 448 with a TR jet ≥ 3 m/s. Of these 448 patients with PH, 47 were identified as "presumptive PAH" and the other 401 as having PH belonging to WHO Groups 2-5. Presumptive PAH represented 1% of screened echocardiograms and 10.5% of those identified to have an elevated TR jet. Of the patients identified as presumptive PAH, 8 underwent further evaluation including a right heart catheterization, where 5 were confirmed to have PAH. Kaplan-Meier analysis revealed 30-day readmission was higher among those classified as PAH. Our data shows that pulmonary hypertension, as defined by TR jet ≥ 3 m/s, is frequently encountered in hospitalized patients undergoing echocardiography for any reason. A careful review of echocardiogram findings and clinical history suggested 10.5% of those with PH (and 1% of all screened patients) may meet the criteria for PAH. Considering PH is a fatal condition which is frequently missed, a hospital screening program seems feasible.