Fluid Restriction in Patients with Pulmonary Arterial Hypertension and Right Heart Failure.

Introduction: In pulmonary arterial hypertension (PAH), right heart (RH) failure is associated with high mortality and poor prognosis. The objective of this cohort study was to assess, whether reduction of fluid intake is associated with RH size and clinical outcome in PAH patients.

Methods: A retrospective, exploratory analysis of patients with invasively diagnosed PAH and signs of fluid retention who were routinely clinically monitored for 8.

Pathogenic SMAD6 variants in patients with idiopathic and complex congenital heart disease associated pulmonary arterial hypertension.

In patients with complex congenital heart disease (CHD) pathogenic SMAD6 variants have been described previously. The aim of this study was to analyze if pathogenic SMAD6 variants also occur in patients with CHD associated with pulmonary arterial hypertension (CHD-APAH) or idiopathic PAH. A PAH gene panel with up to 64 genes including SMAD6 was used to sequence 311 patients with idiopathic PAH (IPAH) and 32 with CHD-APAH.

Automated volumetry of core and peel intrapulmonary vasculature on computed tomography angiography for non-invasive estimation of hemodynamics in patients with pulmonary hypertension (2022 updated hemodynamic definition).

Background: Computed tomography pulmonary angiography (CTPA) is frequently performed in patients with pulmonary hypertension (PH) and may aid non-invasive estimation of pulmonary hemodynamics. We, therefore, investigated automated volumetry of intrapulmonary vasculature on CTPA, separated into core and peel fractions of the lung volume and its potential to differentially reflect pulmonary hemodynamics in patients with pre- and postcapillary PH.

Methods: A retrospective case-control study of 72 consecutive patients with PH according to the 2022 joint guidelines of the European Society of Cardiology and the European Respiratory Society who underwent right heart catheterization (RHC) and CTPA within 7 days between August 2013 and February 2016 at Thoraxklinik at Heidelberg University Hospital (Heidelberg, Germany) was conducted.

Sex-Specific Differences in Echocardiographic Parameters of Risk Stratification in Pulmonary Arterial Hypertension.

Background: In healthy subjects, sex differences in right heart function have been detected for various echocardiographic parameters. The objective of this study was to investigate sex differences in echocardiographic European Society of Cardiology (ESC)/European Respiratory Society (ERS) risk stratification parameters and their impact on survival estimation in patients with pulmonary arterial hypertension (PAH).

Methods: In this retrospective, cross-sectional study with a mean follow-up time of 3.

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.

Long-term oxygen therapy in precapillary pulmonary hypertension - SOPHA study.

Current guidelines recommend oxygen (O) supplementation in patients with pulmonary hypertension (PH), despite scarce data on long-term O therapy (LTOT). The aim of this prospective, randomized, controlled trial was to investigate the effect of LTOT in patients with precapillary PH on exercise capacity, clinical parameters and hemodynamics. Patients with precapillary PH under stable therapy and O desaturations at rest and/or during exercise were randomized to receive LTOT (≥ 16 h/day) or no O (control group) for 12 weeks.

Effect of ambrisentan in patients with systemic sclerosis and mild pulmonary arterial hypertension: long-term follow-up data from EDITA study.

Background: In the EDITA trial, patients with systemic sclerosis (SSc) and mild pulmonary vascular disease (PVD) treated with ambrisentan had a significant decline of pulmonary vascular resistance (PVR) but not of mean pulmonary arterial pressure (mPAP) vs. placebo after six months. The EDITA-ON study aimed to assess long-term effects of open label therapy with ambrisentan vs.

Long-Term Safety, Outcome, and Clinical Effects of Subcutaneous and Intravenous Treprostinil Treatment in Patients with Severe Chronic Pulmonary Arterial Hypertension.

Background: Current guidelines recommend treatment with parenteral prostacyclin analogs in patients with severe pulmonary arterial hypertension (PAH), who have insufficient response to treatment. Real-life data are sought to help physicians in treatment decisions and clinical care of patients.

Objective: This study analyzed safety, clinical effects, and long-term outcomes of subcutaneous (sc) and/or intravenous (iv) treprostinil via different pump systems in consecutive patients with PAH.

Hypochromic red cells as a prognostic indicator of survival among patients with systemic sclerosis screened for pulmonary hypertension.

Background: Patients with systemic sclerosis (SSc) are frequently affected by iron deficiency, particularly those with pulmonary hypertension (PH). The first data indicate the prognostic importance of hypochromic red cells (% HRC) > 2% among patients with PH. Hence, the objective of our study was to investigate the prognostic value of % HRC in SSc patients screened for PH.

Oxygenated hemoglobin as prognostic marker among patients with systemic sclerosis screened for pulmonary hypertension.

Oxygenated hemoglobin (OxyHem) in arterial blood may reflect disease severity in patients with systemic sclerosis (SSc). The aim of this study was to analyze the predictive value of OxyHem in SSc patients screened for pulmonary hypertension (PH). OxyHem (g/dl) was measured by multiplying the concentration of hemoglobin with fractional oxygen saturation in arterialized capillary blood.

Tolerability, safety and survival in patients with severe pulmonary arterial hypertension treated with intravenous epoprostenol (Veletri): a prospective, 6-months, open label, observational, non-interventional study.

Background: Epoprostenol AS (Veletri), a thermostable epoprostenol formulation, provides better drug stability and improved clinical use compared to previous epoprostenol formulations. This study aims to expand clinical experience in the use of Veletri, especially regarding tolerability, safety and survival.

Methods: Pulmonary arterial hypertension (PAH) patients at high risk despite pretreatment with at least double oral combination therapy and with clinical indication for epoprostenol (Veletri) treatment were consecutively included in this prospective, open label, observational, non-interventional study.

Impairment of Inspiratory Muscle Function after COVID-19.

Background: Persistent symptoms after acute coronavirus-disease-2019 (COVID-19) are common, and there is no significant correlation with the severity of the acute disease. In long-COVID (persistent symptoms >4 weeks after acute COVID-19), respiratory symptoms are frequent, but lung function testing shows only mild changes that do not explain the symptoms. Although COVID-19 may lead to an impairment of the peripheral nervous system and skeletal muscles, respiratory muscle function has not been examined in this setting.

The effect of exercise training and physiotherapy on left and right heart function in heart failure with preserved ejection fraction: a systematic literature review.

The impact of exercise training and physiotherapy on heart function and pulmonary circulation parameters in heart failure with preserved ejection fraction (HFpEF) patients is uncertain. Hence, we performed a systematic review of published trials studying physical training in HFpEF population, with a focus on exercise and physiotherapy effect on left ventricular (LV), right ventricular (RV) morphological, functional, and pulmonary circulation parameters. We searched Cochrane Library and MEDLINE/PubMed for trials that evaluated the effect of exercise training and/or physiotherapy in adult HFpEF patients (defined as LVEF ≥ 45%), including publications until March 2021.

Reduction of mRNA Expression in Peripheral Blood of Pulmonary Arterial Hypertension Patients: A Marker for Disease Severity?

Pulmonary arterial hypertension (PAH) can be caused by pathogenic variants in the gene bone morphogenetic protein receptor 2 (BMPR2). While BMPR2 protein expression levels are known to be reduced in the lung tissue of heritable PAH (HPAH) patients, a systematic study evaluating expression in more easily accessible blood samples and its clinical relevance is lacking. Thus, we analyzed the BMPR2 mRNA expression in idiopathic/HPAH patients and healthy controls in blood by quantitative polymerase chain reaction and protein expression by enzyme-linked immunosorbent assay.

Diagnostic accuracy of automated 3D volumetry of cardiac chambers by CT pulmonary angiography for identification of pulmonary hypertension due to left heart disease.

Objectives: To assess diagnostic accuracy of automated 3D volumetry of cardiac chambers based on computed tomography pulmonary angiography (CTPA) for the differentiation of pulmonary hypertension due to left heart disease (group 2 PH) from non-group 2 PH compared to manual diameter measurements.

Methods: Patients with confirmed PH undergoing right heart catheterisation and CTPA within 100 days for diagnostic workup of PH between August 2013 and February 2016 were included in this retrospective, single-centre study. Automated 3D segmentation of left atrium, left ventricle, right atrium and right ventricle (LA/LV/RA/RV) was performed by two independent and blinded radiologists using commercial software.

Utility of Automated Cardiac Chamber Volumetry by Nongated CT Pulmonary Angiography for Detection of Pulmonary Hypertension Using the 2018 Updated Hemodynamic Definition.

Noninvasive tests for pulmonary hypertension (PH) are needed to help select patients for diagnostic right heart catheterization (RHC). CT pulmonary angiography (CTPA) is commonly performed for suspected PH. The purpose of this study was to assess the utility of CTPA-based cardiac chamber volumetric measurements for the diagnosis of PH in comparison with echocardiographic and conventional CTPA parameters, with the 2018 updated hemodynamic definition used as reference.

Prognostic impact of hypochromic erythrocytes in patients with pulmonary arterial hypertension.

Background: Iron deficiency affects up to 50% of patients with pulmonary arterial hypertension (PAH) but iron markers such as ferritin and serum iron are confounded by several non-disease related factors like acute inflammation and diet. The aim of this study was to identify a new marker for iron deficiency and clinical outcome in PAH patients.

Methods: In this single-center, retrospective study we assessed indicators of iron status and clinical parameters specifying the time to clinical worsening (TTCW) and survival in PAH patients at time of initial diagnosis and at 1-year follow-up using univariable and multivariable analysis.

Effect of Supervised Training Therapy on Pulmonary Arterial Compliance and Stroke Volume in Severe Pulmonary Arterial Hypertension and Inoperable or Persistent Chronic Thromboembolic Pulmonary Hypertension.

Background: Pulmonary arterial compliance (PAC) is a prognostic parameter in pulmonary arterial hypertension (PAH) reflecting the elasticity of the pulmonary vessels.

Objectives: The objective of this post hoc analysis of a prospective randomized controlled trial (RCT) was to assess the effect of exercise training on PAC and stroke volume (SV) in patients with PAH and persistent/inoperable chronic thromboembolic pulmonary hypertension (CTEPH).

Method: From the previous RCT, 43 out of 87 patients with severe PAH (n = 29) and CTEPH (n = 14) had complete haemodynamic examinations at baseline and after 15 weeks by right heart catheterization and were analysed (53% female, 79% World Health Organization functional class III/IV, 58% combination therapy, 42% on supplemental oxygen therapy, training group n = 24, and control group n = 19).

Supervised Exercise Training in Patients with Chronic Thromboembolic Pulmonary Hypertension as Early Follow-Up Treatment after Pulmonary Endarterectomy: A Prospective Cohort Study.

Background: Data on exercise training in chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary endarterectomy (PEA) as well as data on clinical and haemodynamic changes shortly after PEA are lacking.

Objective: The objective of this prospective study was to analyse the safety, feasibility, and the effectiveness of combined supervised inpatient rehabilitation in patients with CTEPH directly after PEA.

Methods: CTEPH patients started a 19-week rehabilitation program (3 weeks as inpatients and continued at home for another 16 weeks) with supervised exercise training as follow-up treatment shortly after PEA.