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 already been detected for various echocardiographic parameters.

Research Question: The objective of the study was to investigate sex-differences in echocardiographic ESC/ERS risk stratification parameters and their impact on survival estimation in patients with pulmonary arterial hypertension (PAH).

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

A pulmonary hypertension targeted algorithm to improve referral to right heart catheterization: A machine learning approach.

Background: Pulmonary hypertension (PH) is a pathophysiological problem that may involve several clinical symptoms and be linked to various respiratory and cardiovascular illnesses. Its diagnosis is made invasively by Right Cardiac Catheterization (RHC), which is difficult to perform routinely. Aim of the current study was to develop a Machine Learning (ML) algorithm based on the analysis of anamnestic data to predict the presence of an invasively measured PH.

Efficacy and Safety of Sotatercept Across Ranges of Cardiac Index in Patients with Pulmonary Arterial Hypertension: A Pooled Analysis of PULSAR and STELLAR.

Background: This analysis compared effects of the activin signaling inhibitor, sotatercept, across pulmonary arterial hypertension (PAH) subgroups stratified by baseline cardiac index (CI).

Methods: Pooled data from PULSAR (N=106; NCT03496207) and STELLAR (N=323; NCT04576988) were analyzed using two different CI thresholds, < and ≥ 2.0 L/min/m or 2.

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.

Air travel in patients suffering from pulmonary hypertension-A prospective, multicentre study.

The PEGASUS study is the first multicentric and prospective assessment of the safety of air travel flying in pulmonary hypertension (PH) (NCT03051763). Data of air travel from 60 patients with PH was available. No severe adverse events occurred.

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.

Submaximal, Low-Dose Eccentric vs Traditional Cycling Exercise: Reduced Oxygen Uptake and Pulmonary Artery Pressure Assessed by Echocardiography in Healthy Middle-aged Adults. A Randomized Controlled, Crossover Trial.

Objective: To investigate the ventilatory and circulatory differences between eccentric (ECC) and concentric (CON) cycling exercise at submaximal, low-dose intensity from onset to end-exercise in healthy middle-aged participants.

Design: Randomized controlled crossover trial.

Setting: The participants underwent 1 ECC and 1 CON test according to stepwise incremental exercise protocols at identical, submaximal intensities.

Genetic background of pulmonary (vascular) diseases - how much is written in the codes?

Purpose Of Review: To provide a comprehensive overview of the underlying genetic defects of pulmonary (vascular) diseases and novel treatment avenues.

Recent Findings: Pulmonary arterial hypertension (PAH) is the prime example of a pulmonary vascular disease, which can be caused by genetic mutations in some patients. Germline mutations in the BMPR2 gene and further genes lead to vessel remodelling, increase of pulmonary vascular resistance and onset of heritable PAH.

[Pulmonary hypertension in adults with congenital heart disease in light of the 2022-ESC-PAH guidelines - Part II: Supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesions, left ventricular disorders, univentricular hearts), interventions, intensive care, follow-up, future perspectives].

The number of adults with congenital heart defects (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary hypertension (PH), which may develop early in untreated CHD. Despite timely treatment of CHD, PH not infrequently persists or recurs in older age and is associated with significant morbidity and mortality.

Positive Vasoreactivity Testing in Pulmonary Arterial Hypertension: Therapeutic Consequences, Treatment Patterns, and Outcomes in the Modern Management Era.

Background: Among patients with pulmonary arterial hypertension (PAH), acute vasoreactivity testing during right heart catheterization may identify acute vasoresponders, for whom treatment with high-dose calcium channel blockers (CCBs) is recommended. However, long-term outcomes in the current era remain largely unknown. We sought to evaluate the implications of acute vasoreactivity response for long-term response to CCBs and other outcomes.

The nitric oxide-soluble guanylate cyclase-cGMP pathway in pulmonary hypertension: from PDE5 to soluble guanylate cyclase.

The nitric oxide (NO)-soluble guanylate cyclase (sGC)-cyclic guanosine monophosphate (cGMP) pathway plays a key role in the pathogenesis of pulmonary hypertension (PH). Targeted treatments include phosphodiesterase type 5 inhibitors (PDE5i) and sGC stimulators. The sGC stimulator riociguat is approved for the treatment of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH).

Phenotypes and treatment outcomes in idiopathic pulmonary arterial hypertension patients with comorbidities.

Idiopathic pulmonary arterial hypertension (IPAH) is often diagnosed in elderly patients with many comorbidities. Whereas a clear treatment strategy and risk assessment is recommended for patients with rare classical IPAH, monotherapy with phosphodiesterase type 5 inhibitors or endothelin receptor antagonists followed by regular follow-up and individualised therapy should be used for patients with many cardiopulmonary comorbidities. Here, we focus on these patients with IPAH and comorbidities, present a review of the literature with a focus on recently published work and summarise factors that may help to provide guidance for individualised treatment approaches in such patients.

Randomized Trial of Macitentan/Tadalafil Single-Tablet Combination Therapy for Pulmonary Arterial Hypertension.

Background: Endothelin receptor antagonist (ERA) and phosphodiesterase 5 inhibitor (PDE5i) combination therapy is recommended for low-/intermediate-risk pulmonary arterial hypertension (PAH) patients. A fixed-dose combination of the ERA macitentan and PDE5i tadalafil (M/T FDC) in a once-daily, single tablet would simplify treatment.

Objectives: The multicenter, double-blind, adaptive phase 3 A DUE study investigated the efficacy and safety of M/T FDC vs macitentan 10 mg and vs tadalafil 40 mg monotherapies in PAH patients, including treatment-naïve and prior ERA or PDE5i monotherapy-treated patients.

Oral Treprostinil is Associated with Improved Survival in FREEDOM-EV and its Open-Label Extension.

Introduction: In the event-driven FREEDOM-EV trial, oral treprostinil delayed clinical worsening in patients with pulmonary arterial hypertension (PAH). Open-label extension studies offer additional data about tolerability, efficacy, and survival, especially for those initially assigned placebo. The aim of the current study was to determine if oral treprostinil changed survival when considering the parent and extension study, if treprostinil provides functional benefits for participants initially assigned to placebo, and if the benefits observed for those treated with treprostinil were durable.

[Pulmonary arterial hypertension in congenital heart disease - Part I].

The number of adults with congenital heart disease (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary arterial hypertension (PAH), which may develop early in untreated CHD. Despite timely treatment of CHD, PAH often persists or recurs in older age and is associated with significant morbidity and mortality.

[General measures and management of pulmonary arterial hypertension].

Care of patients with pulmonary arterial hypertension (PAH) needs a multi-facetet concept and measures, including management of adverse reactions, right heart insufficiency as well as information on pregnancy, travels by air, psychosocial support, physical exercise training and prophylaxis by vaccination.Positive study results led to an higher recommendation of specialized exercise training in pulmonary hypertension. Also, the recommendation on iron substitution was amended according to the current evidence.

[Medication treatment in pulmonary arterial hypertension with comorbidities - Which phenotypes and points to consider].

Within the last decade, the age at diagnosis of patients with pulmonary arterial hypertension has increased, which led to a change of the clinical phenoype being associated with more comorbidities. Cluster analyses of registry data have identified cardiac, cardio-pulmonary and classical phenotypes of pulmonary arterial hypertension.Subgroup analyses of randomised controlled trials and registry data indicate, that in patients with pulmonary arterial hypertension and cardiac comorbidities, especially the left-heart phenotype, a closely supervised combination treatment may be considered.