CT scoring system to Defined Thrombus Distribution in Chronic Thromboembolic Pulmonary Hypertension.

Objective: Characterisation of thrombus is important for guiding treatment in chronic thromboembolic pulmonary hypertension (CTEPH). This study presents a novel scoring system for visual assessment of CTEPH on CT pulmonary angiography (CTPA), incorporating both disease location and extent to determine the impact on survival outcomes.

Methods: Patients with CTEPH were identified retrospectively from the ASPIRE registry.

Reaching for the summit in pulmonary arterial hypertension with sotatercept: Results of the ZENITH study.

The ZENITH study recently reported significant benefits from treatment with sotatercept, an activin signaling inhibitor, in patients with pulmonary arterial hypertension. In this Viewpoint, we discuss the findings and implications of this study in the context of the previous PULSAR and STELLAR trials.

NT-proBNP and BNP Testing in Pulmonary Arterial Hypertension: Point-of-Care and Remote Monitoring.

Background And Objectives: Brain natriuretic peptide (BNP) and N-terminal prohormone of BNP (NT-proBNP) are important biomarkers in pulmonary arterial hypertension (PAH). However, results are rarely available at the time of clinical assessment. The reliability of NT-proBNP/BNP point-of-care tests (POCT) in PAH patients and the stability of NT-proBNP in posted blood samples, to simulate remote monitoring, was investigated.

Lung parenchymal and cardiac appearances on computed tomography pulmonary angiography impact survival in chronic thromboembolic pulmonary hypertension: results from the ASPIRE Registry.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is commonly evaluated using computed tomography pulmonary angiography (CTPA). We evaluated the frequency and impact of parenchymal and cardiac abnormalities on survival in CTEPH.

Methods: Patients were identified from the ASPIRE (Assessing the Spectrum of Pulmonary Hypertension Identified at a Referral Centre) Registry.

Selection of patient-reported outcome measures in pulmonary arterial hypertension clinical trials: a systematic review, meta-analysis and health-related quality of life framework.

Introduction: Health-related quality of life (HRQoL) in pulmonary arterial hypertension (PAH) is valued as an outcome measure by patients, clinicians and regulators. The selection of patient-reported outcome measures (PROMs) for measurement of HRQoL in PAH clinical trials lacks systematic evaluation of their suitability, accuracy and reliability.

Methods: We report a systematic review (PROSPERO ID: CRD42024484021) following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines of PROMs selected in PAH clinical trials.

Diagnostic MicroRNA Signatures to Support Classification of Pulmonary Hypertension.

Background: Patients with pulmonary hypertension (PH) are classified based on disease pathogenesis and hemodynamic drivers. Classification informs treatment. The heart failure biomarker NT-proBNP (N-terminal pro-B-type natriuretic peptide) is used to help inform risk but is not specific to PH or sub-classification groups.

Mean pulmonary artery pressure prediction with explainable multi-view cardiovascular magnetic resonance cine series deep learning model.

Background: Pulmonary hypertension (PH) is a heterogeneous condition and regardless of etiology impacts negatively on survival. Diagnosis of PH is based on hemodynamic parameters measured invasively at right heart catheterization (RHC); however, a non-invasive alternative would be clinically valuable. Our aim was to estimate RHC parameters non-invasively from cardiac magnetic resonance (MR) data using deep learning models and to identify key contributing imaging features.

Prognostic relevance of exercise pulmonary hypertension: results of the multicentre PEX-NET Clinical Research Collaboration.

Background: Exercise pulmonary hypertension (PH) was defined by a mean pulmonary arterial pressure (mPAP)/cardiac output (CO) slope >3 mmHg·min·L between rest and exercise in the 2022 European Society of Cardiology/European Respiratory Society PH guidelines. However, large, multicentre studies on the prognostic relevance of exercise haemodynamics and its added value to resting haemodynamics are missing.

Patients And Methods: The PEX-NET (Pulmonary Haemodynamics during Exercise Network) registry enrolled patients who underwent clinically indicated right heart catheterisations both at rest and ergometer exercise from 23 PH centres worldwide.

Pulmonary vascular manifestations of hereditary haemorrhagic telangiectasia.

Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant, multisystem disorder that manifests with a spectrum of disease including cardiopulmonary complications. HHT is characterised by aberrant signalling via the transforming growth factor β (TGFβ) pathway, with loss of vascular integrity, angiogenesis and vascular dysplasia. The disease has an estimated prevalence of 1 in 5000 persons and the penetrance increases with increasing age.

Do Hemodynamic Definitions of Chronic Thromboembolic Pulmonary Hypertension Distinguish between Distinct Phenotypes of Chronic Thromboembolic Pulmonary Disease?

Chronic thromboembolic pulmonary disease (CTEPD) is defined by chronic organized thrombi in the pulmonary circulation without or with pulmonary hypertension. The current definition of chronic thromboembolic pulmonary hypertension (CTEPH) has adopted lower mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) thresholds. Our aim was to identify its impact on the characterization of patients with CTEPD.

The spectrum of systemic sclerosis-associated pulmonary hypertension: Insights from the ASPIRE registry.

Background: There are limited data assessing the spectrum of systemic sclerosis-associated pulmonary hypertension (PH).

Methods: Data for 912 systemic sclerosis patients assessed between 2000 and 2020 were retrieved from the Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre (ASPIRE) registry and classified based on 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines and multimodality investigations.

Results: Reduction in pulmonary vascular resistance (PVR) diagnostic threshold to >2WU resulted in a 19% increase in precapillary PH diagnoses.

Unenhanced computed tomography as a diagnostic tool in suspected pulmonary hypertension: a retrospective cross-sectional pilot study.

Background: Computed tomography pulmonary angiography (CTPA) has been proposed to be diagnostic for pulmonary hypertension (PH) in multiple studies. However, the utility of the unenhanced CT measurements diagnosing PH has not been fully assessed. This study aimed to assess the diagnostic utility and reproducibility of cardiac and great vessel parameters on unenhanced computed tomography (CT) in suspected pulmonary hypertension (PH).

Pulmonary Hypertension: Intensification and Personalization of Combination Rx (PHoenix): A phase IV randomized trial for the evaluation of dose-response and clinical efficacy of riociguat and selexipag using implanted technologies.

Approved therapies for the treatment of patients with pulmonary arterial hypertension (PAH) mediate pulmonary vascular vasodilatation by targeting distinct biological pathways. International guidelines recommend that patients with an inadequate response to dual therapy with a phosphodiesterase type-5 inhibitor (PDE5i) and endothelin receptor antagonist (ERA), are recommended to either intensify oral therapy by adding a selective prostacyclin receptor (IP) agonist (selexipag), or switching from PDE5i to a soluble guanylate-cyclase stimulator (sGCS; riociguat). The clinical equipoise between these therapeutic choices provides the opportunity for evaluation of individualized therapeutic effects.

Improving Prognostication in Pulmonary Hypertension Using AI-quantified Fibrosis and Radiologic Severity Scoring at Baseline CT.

Background There is clinical need to better quantify lung disease severity in pulmonary hypertension (PH), particularly in idiopathic pulmonary arterial hypertension (IPAH) and PH associated with lung disease (PH-LD). Purpose To quantify fibrosis on CT pulmonary angiograms using an artificial intelligence (AI) model and to assess whether this approach can be used in combination with radiologic scoring to predict survival. Materials and Methods This retrospective multicenter study included adult patients with IPAH or PH-LD who underwent incidental CT imaging between February 2007 and January 2019.

Systematic pulmonary embolism follow-up increases diagnostic rates of chronic thromboembolic pulmonary hypertension and identifies less severe disease: results from the ASPIRE Registry.

Background: Diagnostic rates and risk factors for the subsequent development of chronic thromboembolic pulmonary hypertension (CTEPH) following pulmonary embolism (PE) are not well defined.

Methods: Over a 10-year period (2010-2020), consecutive patients attending a PE follow-up clinic in Sheffield, UK (population 554 600) and all patients diagnosed with CTEPH at a pulmonary hypertension (PH) referral centre in Sheffield (referral population estimated 15-20 million) were included.

Results: Of 1956 patients attending the Sheffield PE clinic 3 months following a diagnosis of acute PE, 41 were diagnosed with CTEPH with a cumulative incidence of 2.

Blood DNA methylation profiling identifies cathepsin Z dysregulation in pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is characterised by pulmonary vascular remodelling causing premature death from right heart failure. Established DNA variants influence PAH risk, but susceptibility from epigenetic changes is unknown. We addressed this through epigenome-wide association study (EWAS), testing 865,848 CpG sites for association with PAH in 429 individuals with PAH and 1226 controls.

From the microscopic to the macroscopic: clinical-radiological-pathological correlation in pulmonary hypertension.

https://bit.ly/3RtiFVK

Clinical-radiological-pathological correlation in chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and potentially life-threatening complication of acute pulmonary embolism. It is characterised by persistent fibro-thrombotic pulmonary vascular obstructions and elevated pulmonary artery pressure leading to right heart failure. The diagnosis is based on two steps, as follows: 1) suspicion based on symptoms, echocardiography and ventilation/perfusion scan and 2) confirmation with right heart catheterisation, computed tomography pulmonary angiography and, in most cases, digital subtraction angiography.

Clinical-radiological-pathological correlation in pulmonary arterial hypertension.

Pulmonary hypertension (PH) is defined by the presence of a mean pulmonary arterial pressure >20 mmHg. Current guidelines describe five groups of PH with shared pathophysiological and clinical features. In this paper, the first of a series covering all five PH classification groups, the clinical, radiological and pathological features of pulmonary arterial hypertension (PAH) will be reviewed.

The Left Atrial Area Derived Cardiovascular Magnetic Resonance Left Ventricular Filling Pressure Equation Shows Superiority over Integrated Echocardiography.

: Evaluating left ventricular filling pressure (LVFP) plays a crucial role in diagnosing and managing heart failure (HF). While traditional assessment methods involve multi-parametric transthoracic echocardiography (TTE) or right heart catheterisation (RHC), cardiovascular magnetic resonance (CMR) has emerged as a valuable diagnostic tool in HF. This study aimed to assess a simple CMR-derived model to estimate pulmonary capillary wedge pressure (PCWP) in a cohort of patients with suspected or proven heart failure and to investigate its performance in risk-stratifying patients.

Surgery and Anesthesia in Patients with Pulmonary Hypertension.

Pulmonary hypertension is characterized by right ventricular impairment and a reduced ability to compensate for hemodynamic insults. Consequently, surgery can be challenging but is increasingly considered in view of available specific therapies and improved longer term survival. Optimal management requires a multidisciplinary patient-centered approach involving surgeons, anesthetists, pulmonary hypertension clinicians, and intensivists.

Establishing minimally important differences for cardiac MRI end-points in pulmonary arterial hypertension.

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".

Assessment of Right Ventricular Function-a State of the Art.

Purpose Of Review: The right ventricle (RV) has a complex geometry and physiology which is distinct from the left. RV dysfunction and failure can be the aftermath of volume- and/or pressure-loading conditions, as well as myocardial and pericardial diseases.

Recent Findings: Echocardiography, magnetic resonance imaging and right heart catheterisation can assess RV function by using several qualitative and quantitative parameters.

Non-invasive detection of severe PH in lung disease using magnetic resonance imaging.

Introduction: Severe pulmonary hypertension (mean pulmonary artery pressure ≥35 mmHg) in chronic lung disease (PH-CLD) is associated with high mortality and morbidity. Data suggesting potential response to vasodilator therapy in patients with PH-CLD is emerging. The current diagnostic strategy utilises transthoracic Echocardiography (TTE), which can be technically challenging in some patients with advanced CLD.

Computational platform for doctor-artificial intelligence cooperation in pulmonary arterial hypertension prognostication: a pilot study.

Background: Pulmonary arterial hypertension (PAH) is a heterogeneous and complex pulmonary vascular disease associated with substantial morbidity. Machine-learning algorithms (used in many PAH risk calculators) can combine established parameters with thousands of circulating biomarkers to optimise PAH prognostication, but these approaches do not offer the clinician insight into what parameters drove the prognosis. The approach proposed in this study diverges from other contemporary phenotyping methods by identifying patient-specific parameters driving clinical risk.