Mono and combination therapies in pulmonary arterial hypertension patients with comorbidities: A COMPERA analysis.

Aims: Pulmonary arterial hypertension (PAH) is often diagnosed in elderly patients with comorbidities. Although initial monotherapy is recommended for these patients, the value of combination therapy remains unclear. Here, we compare the efficacy of initial monotherapy and combination therapy in PAH patients with cardiovascular comorbidities.

Comparison of the Equivalence of Aspergillus Antigen and PCR Results Between Non-Directed Bronchial Lavage and Bronchoalveolar Lavage-A Prospective Exploratory Pilot Study in Critically Ill Patients.

Background: Obtaining non-directed samples from the upper bronchial tree is easier to perform and poses fewer risks for critically ill patients than deep bronchoalveolar lavage (BAL). Since invasive pulmonary aspergillosis is associated with a high mortality in critically ill patients, timely diagnosis and rapid initiation of treatment are of utmost importance.

Objectives: The objective of this study was to compare Galactomannan (GM) testing by Enzyme Immunoassay (EIA), GM Lateral Flow Assay (LFA) and the detection of Aspergillus DNA by Polymerase Chain Reaction (PCR) between directed BAL and non-directed bronchial lavage (BL) in critically ill patients.

Sex-specific Genetic Determinants of Right Ventricular Structure and Function.

Rationale: While sex differences in right heart phenotypes have been observed, the molecular drivers remain unknown.

Objectives: To provide biological insights into sex differences in the structure and function of the right ventricle (RV) using common genetic variation.

Methods: RV phenotypes were obtained from cardiac magnetic resonance imaging in 18,156 women and 16,171 men from the UK Biobank.

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.

Right ventricular strain to systolic pulmonary artery pressure ratio in response to treatment of pulmonary arterial hypertension.

https://bit.ly/4cRtREC.

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.

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.

[Targeted therapy for pulmonary arterial hypertension in patients without comorbidities].

The 2022 guidelines on pulmonary hypertension from the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) provide therapeutic strategies that account for the variability in the clinical presentation of newly diagnosed patients. We summarize treatment recommendations for pulmonary arterial hypertension (PAH) in patients without significant comorbidities, particularly for idiopathic, hereditary, drug/toxin-induced, or connective tissue disease-associated PAH. In this group of patients, multidimensional assessments for short-term mortality risk guide initial treatment decisions and treatment decisions during follow-up.

[Pathophysiology, diagnosis, prognosis and treatment of pulmonary hypertension associated with chronic lung disease].

The pathophysiology of pulmonary hypertension associated with chronic lung disease (PH-CLD) is complex, multifactorial, and not consistent among pulmonary diseases. However, pulmonary vasculopathy triggered by various factors, such as chronic alveolar hypoxia or cigarette smoking, seems to play a central role in the pathogenesis of PH-CLD. While the initial workup of PH-CLD is usually complicated by an overlap of symptoms of PH and the underlying lung disease, PH-CLD should be considered when there is a discrepancy between symptoms (especially exertional dyspnea) and pulmonary function tests.

Blood T cell phenotypes correlate with fatigue severity in post-acute sequelae of COVID-19.

Purpose: Post-acute sequelae of COVID-19 (PASC) affect approximately 10% of convalescent patients. The spectrum of symptoms is broad and heterogeneous with fatigue being the most often reported sequela. Easily accessible blood biomarkers to determine PASC severity are lacking.

Psychological risk factors for Long COVID and their modification: study protocol of a three-arm, randomised controlled trial (SOMA.COV).

Background: Growing evidence suggests that in addition to pathophysiological, there are psychological risk factors involved in the development of Long COVID. Illness-related anxiety and dysfunctional symptom expectations seem to contribute to symptom persistence.

Aims: With regard to the development of effective therapies, our primary aim is to investigate whether symptoms of Long COVID can be improved by a targeted modification of illness-related anxiety and dysfunctional symptom expectations.

Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis.

Objectives: Pulmonary arterial hypertension (PAH) occurs in various connective tissue diseases (CTDs). We sought to assess contemporary treatment patterns and survival of patients with various forms of CTD-PAH.

Methods: We analysed data from COMPERA, a European pulmonary hypertension registry, to describe treatment strategies and survival in patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types of CTD.

Residual risk identified in routine noninvasive follow-up assessments in pulmonary arterial hypertension.

Background: The 2022 ESC/ERS guidelines on pulmonary hypertension recommend noninvasive risk assessments based on three clinical variables during follow-up in patients with pulmonary arterial hypertension (PAH). We set out to test whether residual risk can be captured from routinely measured noninvasive clinical variables during follow-up in PAH.

Methods: We retrospectively studied 298 incident PAH patients from a German pulmonary hypertension centre who underwent routine noninvasive follow-up assessments including exercise testing, echocardiography, electrocardiography, pulmonary function testing and biochemistry.

Trends in COVID-19-associated mortality in patients with pulmonary hypertension: a COMPERA analysis.

https://bit.ly/42OMsfj

Clinical phenotyping of plasma thrombospondin-2 reveals relationship to right ventricular structure and function in pulmonary hypertension.

Background: Converging evidence from proteogenomic analyses prioritises thrombospondin-2 (TSP2) as a potential biomarker for idiopathic or heritable pulmonary arterial hypertension (PAH). We aimed to assess TSP2 levels in different forms of pulmonary hypertension (PH) and to define its clinical phenotype.

Methods: Absolute concentrations of TSP2 were quantified in plasma samples from a prospective single-centre cohort study including 196 patients with different forms of PH and 16 disease controls (suspected PH, but normal resting pulmonary haemodynamics).

Transcription factors in the pathogenesis of pulmonary arterial hypertension-Current knowledge and therapeutic potential.

Pulmonary arterial hypertension (PAH) is a disease characterized by elevated pulmonary vascular resistance and pulmonary artery pressure. Mortality remains high in severe cases despite significant advances in management and pharmacotherapy. Since currently approved PAH therapies are unable to significantly reverse pathological vessel remodeling, novel disease-modifying, targeted therapeutics are needed.

[Intimal pulmonary sarcoma: A case report of a rare differential diagnosis of chronic thromboembolic pulmonary hypertension].

Pulmonary angiosarcoma is a rare and malignant disease of the blood vessels. Initially, it can be misdiagnosed as chronic thromboembolic hypertension (CTEPH). In CTEPH, there is increased pressure and resistance of the pulmonary arteries following persistent obstruction of pulmonary circulation from (recurrent) thromboembolism despite adequate anticoagulative treatment.

[Sarcoidosis as prime example of a granulomatous disease].

Sarcoidosis is the most frequent immunologically related granulomatous disease and can serve as a model for understanding diseases within this category. The evidence on the diagnostics and treatment is so far limited. It is therefore all the more important that two new and significant guidelines on diagnosis and treatment of sarcoidosis were published during the last 2 years.

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis.

Background: Among patients meeting diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH), there is an emerging lung phenotype characterised by a low diffusion capacity for carbon monoxide (DLCO) and a smoking history. The present study aimed at a detailed characterisation of these patients.

Methods: We analysed data from two European pulmonary hypertension registries, COMPERA (launched in 2007) and ASPIRE (from 2001 onwards), to identify patients diagnosed with IPAH and a lung phenotype defined by a DLCO of less than 45% predicted and a smoking history.

Respiratory muscle dysfunction in long-COVID patients.

Purpose: Symptoms often persistent for more than 4 weeks after COVID-19-now commonly referred to as 'Long COVID'. Independent of initial disease severity or pathological pulmonary functions tests, fatigue, exertional intolerance and dyspnea are among the most common COVID-19 sequelae. We hypothesized that respiratory muscle dysfunction might be prevalent in persistently symptomatic patients after COVID-19 with self-reported exercise intolerance.

Prognostic value of improvement endpoints in pulmonary arterial hypertension trials: A COMPERA analysis.

Background: The prognostic value of improvement endpoints that have been used in clinical trials of treatments for pulmonary arterial hypertension (PAH) needs to be further investigated.

Methods: Using the COMPERA database, we evaluated the prognostic value of improvements in functional class (FC) and absolute or relative improvements in 6-min walking distance (6MWD) and N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP). In addition, we investigated multicomponent endpoints based on prespecified improvements in FC, 6MWD and NT-proBNP that have been used in recent PAH trials.

[Organ-specific sequelae of COVID-19 in adults].

Organ-specific sequelae after COVID-19 occur frequently and are highly diverse in their features. Sequelae and symptoms persisting for more than four weeks after COVID-19 define the condition "long COVID."Organ-specific sequelae of COVID-19 generally occur more often after severe disease.