[Pulmonary hypertension associated with left heart disease (group 2)].

Pulmonary hypertension associated with left heart disease (PH-LHD) corresponds to group two of pulmonary hypertension according to clinical classification. Haemodynamically, this group includes isolated post-capillary pulmonary hypertension (IpcPH) and combined post- and pre-capillary pulmonary hypertension (CpcPH). PH-LHD is defined by an mPAP > 20 mmHg and a PAWP > 15 mmHg, pulmonary vascular resistance (PVR) with a cut-off value of 2 Wood Units (WU) is used to differentiate between IpcPH and CpcPH.

Intravenous Treprostinil in Severe Inoperable Chronic Thromboembolic Pulmonary Hypertension Using Implantable Pumps-Single-Center Experience over More Than a Decade.

The management of chronic thromboembolic pulmonary hypertension has significantly changed over the last decade with the availability of both specific therapies and interventional treatments. In parallel, implantable pumps for intravenous administration of treprostinil have broadened the spectrum of continuous prostanoid infusion. We evaluated the course of 17 consecutive patients with inoperable chronic thromboembolic pulmonary hypertension treated with treprostinil by means of an implantable infusion pump between 2011 and 2023 at our center.

The risk profile change in patients with severe chronic thromboembolic pulmonary hypertension treated with subcutaneous treprostinil.

Chronic thromboembolic pulmonary hypertension (CTEPH) is successfully treatable with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty, and medical therapy. Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management risk score (RRS) is able to predict long-term outcome in inoperable patients or in patients with residual PH after surgery. We performed a post hoc analysis of RRS in patients who were enrolled in the CTREPH study (NCT01416636), a randomized, double-blind clinical trial comparing high-dose and low-dose subcutaneous (SC) treprostinil in patients with severe CTEPH that was classified by an interdisciplinary CTEPH team as nonoperable, or as persistent or recurrent pulmonary hypertension after PEA.

The combination of supervised and unsupervised learning based risk stratification and phenotyping in pulmonary arterial hypertension-a long-term retrospective multicenter trial.

Background: Accurate risk stratification in pulmonary arterial hypertension (PAH), a devastating cardiopulmonary disease, is essential to guide successful therapy. Machine learning may improve risk management and harness clinical variability in PAH.

Methods: We conducted a long-term retrospective observational study (median follow-up: 67 months) including 183 PAH patients from three Austrian PAH expert centers.

Riociguat in pulmonary hypertension and heart failure with preserved ejection fraction: the haemoDYNAMIC trial.

Aims: The presence of pulmonary hypertension (PH) severely aggravates the clinical course of heart failure with preserved ejection fraction (HFpEF). To date, neither established heart failure therapies nor pulmonary vasodilators proved beneficial. This study investigated the efficacy of chronic treatment with the oral soluble guanylate cyclase stimulator riociguat in patients with PH-HFpEF.

Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry.

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice.

Methods: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat.

Riociguat treatment in patients with pulmonary arterial hypertension: Final safety data from the EXPERT registry.

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice.

Methods: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat.

Long-term experience with implantable infusion pumps for intravenous treprostinil in pulmonary arterial hypertension-procedural safety and system-related complications.

Implantable pumps for intravenous treprostinil provide a promising option to overcome drawbacks of parenteral prostanoid administration with external pumps in pulmonary hypertension. We retrospectively analyzed 85 patients undergoing implantation in a single center since 2010. In our cohort, serious complications were rare, and flow rate increase over time warrants careful monitoring.

Rapid Switch From Subcutaneous to Intravenous Treprostinil in Precapillary Pulmonary Hypertension by Pump Implantation.

Limited data are available on the transition from subcutaneous to intravenous prostacyclin in precapillary pulmonary hypertension. We performed a retrospective analysis of all patients who were switched from subcutaneous to intravenous treprostinil with an implantable infusion pump. We included 85 consecutive, clinically stable patients (mean age 66 years and range 16-85), who had been treated with subcutaneous treprostinil for mean 9 months (range 1-78) before pump implantation.

Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension (CTREPH): a double-blind, phase 3, randomised controlled trial.

Background: Treprostinil, a prostacyclin analogue, is effective for the treatment of pulmonary arterial hypertension. However, information is scarce regarding treprostinil for treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to examine the efficacy and safety of subcutaneous treprostinil in this setting.

The crucial impact of iron deficiency definition for the course of precapillary pulmonary hypertension.

Imbalances of iron homeostasis are associated with an adverse clinical outcome of pulmonary hypertension (PH). Herein, we aimed to analyze the impact of iron deficiency (ID) in a real-life PH patient cohort according to different currently used ID definitions. In a retrospective study including 153 precapillary PH patients followed over a mean period of five years, iron deficiency was assessed according to five clinical definitions used in previous trials.

Regression of left atrial diameter after kidney transplantation is associated with prolonged survival: an observational study.

Renal transplantation reduces the dramatically elevated risk of cardiovascular death in dialysis patients. We previously showed that left atrial diameter before transplantation predicts cardiovascular and overall mortality. Now, we investigated the association of changes in cardiac morphology after transplantation and mortality.

Intravenous treprostinil infusion via a fully implantable pump for pulmonary arterial hypertension.

Objectives: Parenteral prostanoids infused via external pumps are well-established pulmonary arterial hypertension (PAH) treatments. However, local side-effects and systemic infections restrict their use. The purpose of this study was to investigate the safety of a fully implantable treprostinil infusion pump (LENUS Pro) in patients with PAH.

Evaluation of the pharmacoDYNAMIC effects of riociguat in subjects with pulmonary hypertension and heart failure with preserved ejection fraction : Study protocol for a randomized controlled trial.

Background: The presence of pulmonary hypertension (PH) severely aggravates the clinical course of heart failure with preserved ejection fraction (HFPEF) resulting in substantial morbidity and mortality. So far, neither established heart failure therapies nor pulmonary vasodilators have proven to be effective for this condition. Riociguat (Adempas®, BAY 63-2521), a stimulator of soluble guanylate cyclase, is a novel pulmonary and systemic vasodilator that has been approved for the treatment of precapillary forms of PH.

Acute hemodynamic effects of riociguat in patients with pulmonary hypertension associated with diastolic heart failure (DILATE-1): a randomized, double-blind, placebo-controlled, single-dose study.

Background: Deficient nitric oxide-soluble guanylate cyclase-cyclic guanosine monophosphate signaling results from endothelial dysfunction and may underlie impaired cardiac relaxation in patients with heart failure with preserved left ventricular ejection fraction (HFpEF) and pulmonary hypertension (PH). The acute hemodynamic effects of riociguat, a novel soluble guanylate cyclase stimulator, were characterized in patients with PH and HFpEF.

Methods: Clinically stable patients receiving standard HF therapy with a left ventricular ejection fraction > 50%, mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg, and pulmonary arterial wedge pressure (PAWP) > 15 mm Hg at rest were randomized to single oral doses of placebo or riociguat (0.

Real-time 3D echocardiographic quantification of left atrial volume: multicenter study for validation with CMR.

Objectives: We studied in a multicenter setting the accuracy and reproducibility of 3-dimensional echocardiography (3DE)-derived measurements of left atrial volume (LAV) using new, dedicated volumetric software, side by side with 2-dimensional echocardiography (2DE), using cardiac magnetic resonance (CMR) imaging as a reference.

Background: Increased LAV is associated with adverse cardiovascular outcomes. Although LAV measurements are routinely performed using 2DE, this methodology is limited because it is view dependent and relies on geometric assumptions regarding left atrial shape.

Assessment of right ventricular function using echocardiographic speckle tracking of the tricuspid annular motion: comparison with cardiac magnetic resonance.

Background: Assessment of right ventricular (RV) function is difficult due to the complex shape of this chamber. Tricuspid annular plane systolic excursion (TAPSE) measured with M-mode echocardiography is frequently used as an index of RV function. However, its accuracy may be limited by ultrasound beam misalignment.

Three-dimensional analysis of regional left ventricular endocardial curvature from cardiac magnetic resonance images.

Purpose: Left ventricular (LV) remodeling is usually assessed using global changes in LV volume. We hypothesized that three-dimensional analysis of regional endocardial curvature from magnetic resonance images could provide clinically useful information on localized LV remodeling. We tested this approach by investigating regional differences in endocardial curvature in normal and hypokinetic ventricles.

Multimodality comparison of quantitative volumetric analysis of the right ventricle.

Objectives: We undertook volumetric analysis of the right ventricle (RV) by real-time 3-dimensional echocardiography (RT3DE), cardiac magnetic resonance (CMR), and cardiac computed tomography (CCT) on images obtained in RV-shaped phantoms and in patients with a wide range of RV geometry.

Background: Assessment of the RV by 2-dimensional (2D) echocardiography remains challenging due to its unique geometry and limitations of the current analysis techniques. RT3DE, CMR, and CCT, which can quantify RV volumes, promise to overcome the limitations of 2D echocardiography.

Quantitative evaluation of regional left ventricular function using three-dimensional speckle tracking echocardiography in patients with and without heart disease.

Although 2-dimensional (2D) speckle tracking echocardiography has been shown to be useful in the assessment of regional left ventricular function, it is limited by the assumption that speckles can be tracked frame-to-frame within the imaging plane, even though the cardiac motion is 3-dimensional (3D). Our goal was to evaluate new 3D-speckle tracking echocardiographic (STE) software by (1) comparing the regional wall motion measurements against 2D-STE images, and (2) testing its ability to identify regional wall motion abnormalities. The 2D images and real-time 3D data sets (Toshiba) obtained from 32 subjects were analyzed to measure segmental radial and longitudinal displacements and rotation, as well as the radial, longitudinal, and circumferential strains.

Quantification of left ventricular volumes using three-dimensional echocardiographic speckle tracking: comparison with MRI.

Aims: Although the utility of two-dimensional (2D) speckle tracking echocardiography (STE) to quantify left ventricular (LV) volume has been demonstrated, this methodology is limited by foreshortened views, geometric modelling, and the assumption that speckles can be tracked from frame to frame, despite their out of plane motion. To circumvent these limitations, a three-dimensional (3D) speckle tracking algorithm was recently developed. Our goal was to evaluate the accuracy of the new 3D-STE side by side with 2D-STE using cardiac magnetic resonance (CMR) as a reference.

Real-time 3-dimensional echocardiographic quantification of left ventricular volumes: multicenter study for validation with magnetic resonance imaging and investigation of sources of error.

Objectives: We sought to study: 1) the accuracy and reproducibility of real-time 3-dimensional echocardiographic (RT3DE) analysis of left ventricular (LV) volumes in a multicenter setting, 2) interinstitutional differences in relationship with the investigators' specific experience, and 3) potential sources of volume underestimation.

Background: Reproducibility and accuracy of RT3DE evaluation of LV volumes has not been validated in multicenter studies, and LV volumes have been reported to be underestimated compared to cardiac magnetic resonance (CMR) standard.

Methods: A total of 92 patients with a wide range of ejection fractions underwent CMR and RT3DE imaging at 4 different institutions.

Volumetric analysis of regional left ventricular function with real-time three-dimensional echocardiography: validation by magnetic resonance and clinical utility testing.

Background: Quantitative information on regional left ventricular volumes from real-time three-dimensional echocardiographic (RT3DE) images has significant clinical potential but needs validation.

Aim: To validate these measurements against cardiac magnetic resonance (CMR) and test the feasibility of automated detection of regional wall motion (RWM) abnormalities from RT3DE data.

Methods: RT3DE (Philips) and CMR (Siemens) images were obtained from 31 patients and analysed by using prototype software to semiautomatically calculate indices of regional left ventricular function, which were compared between RT3DE and CMR (linear regression, Bland-Altman).