Gabor Kovacs

Gabor Kovacs
Ludwig Boltzmann Institute for Lung Vascular Research

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200
Publications
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Publications

Publications (200)
Article
Background & aims Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are severe pulmonary vascular complications of chronic liver disease and strongly associated with morbidity and mortality. The prevalence of these complications is relatively high in patients evaluated for liver transplantation, however it is virtually unknown i...
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Background Mean pulmonary artery wedge pressure (PAWP) represents a right heart catheter (RHC) surrogate measure for mean left atrial (LA) pressure and is crucial for the clinical classification of pulmonary hypertension (PH). Hypothesizing that PAWP is related to acceleration of blood throughout the LA, we investigated whether an adequately introd...
Article
Rationale: Pulmonary hypertension (PH) is a common, yet severe comorbidity in interstitial lung diseases (ILD) such as pulmonary fibrosis (PF), with limited treatment options. Excessive vascular fibrosis and inflammation are often present in PH, but the underlying mechanisms are still not well understood. Objective: To identify a novel functiona...
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The cardiopulmonary hemodynamic profile observed during exercise may identify patients with early stage pulmonary vascular and primary cardiac diseases, and is used clinically to inform prognosis. However, a standardised approach to interpreting hemodynamics is lacking. We performed a systematic literature search according to PRISMA guidelines to i...
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Purpose Elevated mean pulmonary arterial pressure (mPAP), or pulmonary hypertension (pH), is associated with vortical blood flow along the main pulmonary artery. We present and validate a method for automated detection and tracking of the PH-related vortex from magnetic resonance 4D flow data that allows estimation of mPAP. Methods The proposed me...
Article
Background Severe pulmonary hypertension (PH) is prognostically highly relevant in patients with COPD. The criteria for severe PH have been defined based on hemodynamic thresholds in right heart catheterization. Research question Can non-invasive clinical tools predict severe PH in COPD patients? How does the mortality risk change with increasing...
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Objectives: Limited cutaneous systemic sclerosis (lcSSc) is characterised by vasculopathy contributing to vascular apoptosis, structural and functional changes. The aim of this study was to investigate parameters of endothelial dysfunction and their association to clinical events in lcSSc patients with early-stage vasculopathy. Methods: Patients...
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Systemic sclerosis (SSc) is an intractable autoimmune disease characterized by vasculopathy and organ fibrosis. Autologous hematopoietic stem cell transplantation (AHSCT) should be considered for the treatment of selected patients with rapid progressive SSc at high risk of organ failure. It, however, remains elusive whether immunosuppressive therap...
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Zusammenfassung Die pulmonale Hypertonie (PH) wird laut aktuellem 6. „World Symposium on Pulmonary Hypertension“ (WSPH) in 5 Gruppen unterteilt, die pulmonalarterielle Hypertonie (PAH, Gruppe 1), die PH bei Linksherzerkrankungen (Gruppe 2), die PH bei Lungenerkrankungen und/oder Hypoxie (Gruppe 3), die chronisch thrombembolische PH (CTEPH, Gruppe 4...
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The diagnosis and management of pulmonary arterial hypertension (PAH) includes several advances, such as broader recognition of extra-pulmonary vascular organ system involvement, validated point-of-care clinical assessment tools, and a focus on early-initiation of multiple pharmacotherapeutics in appropriate patients. Indeed, a principal goal in PA...
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Background - Approximately 25% of patients with pulmonary arterial hypertension (PAH) have been found to harbor rare mutations in disease-causing genes. To identify missing heritability in PAH we integrated deep phenotyping with whole-genome sequencing data using Bayesian statistics. Methods - We analyzed 13,037 participants enrolled in the NIHR Bi...
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Aims This prospective, randomized, controlled, multicentre study aimed to evaluate efficacy and safety of exercise training in patients with pulmonary arterial (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Methods and results For the first time a specialized PAH/CTEPH rehabilitation programme was implemented in 11 centres across...
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Objectives: Longitudinal hemodynamic follow-up is important in the management of pulmonary hypertension (PH). This study aimed to evaluate the potential of MR 4-dimensional (4D) flow imaging to predict changes in the mean pulmonary arterial pressure (mPAP) during serial investigations. Methods: Forty-four adult patients with PH or at risk of dev...
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Background: Pulmonary hemodynamics during exercise may reveal early pulmonary vascular disease and be of clinical and prognostic relevance in systemic sclerosis (SSc). We aimed to assess the prognostic relevance of exercise pulmonary resistances in patients with SSc with no or mildly increased mean pulmonary arterial pressure (mPAP). Research que...
Preprint
Full-text available
Background Approximately 25% of patients with pulmonary arterial hypertension (PAH) have been found to harbor rare mutations in disease-causing genes. To identify missing heritability in PAH we integrated deep phenotyping with whole-genome sequencing data using Bayesian statistics. Methods We analyzed 13,037 participants enrolled in the NIHR BioRe...
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Background In pulmonary hypertension subgroups, elevated pulmonary vascular resistance (PVR) of 3·0 Wood units or more is associated with poor prognosis. However, the spectrum of PVR risk in pulmonary hypertension is not known. To address this area of uncertainty, we aimed to analyse the relationship between PVR and adverse clinical outcomes in pul...
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Even mild pulmonary hypertension (PH) is associated with increased mortality and morbidity in chronic obstructive pulmonary disease (COPD) patients. However, the underlying mechanisms remain elusive; therefore, specific and efficient treatment options are not available. Therapeutic approaches tested in the clinical setting, including long‐term oxyg...
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Background: Systemic sclerosis (SSc) is a severe rheumatic disease of the interstitial tissue, in which heart and lung involvement can lead to disease-specific mortality. Our study tests the hypothesis that in addition to established prognostic factors, cardiopulmonary exercise testing (CPET) parameters, particularly peak oxygen uptake (peakVO2) a...
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Objectives: Recently, rare heterozygous mutations in GDF2 were identified in patients with pulmonary arterial hypertension (PAH). GDF2 encodes the circulating bone morphogenetic protein, BMP9, which is a ligand for the BMP type 2 receptor (BMPR2). Here we determine the functional impact of GDF2 mutations and characterised plasma BMP9 and BMP10 lev...
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Zusammenfassung Die Ende 2015 veröffentlichten ESC/ERS-Leitlinien und andere internationale Empfehlungen definierten die pulmonale Hypertonie (PH) bisher durch einen invasiv gemessenen mittleren pulmonal arteriellen Druck (mPAP) ≥ 25 mmHg in Ruhe. Auf dem 6th World Symposium on Pulmonary Hypertension in Nizza wurde eine Modifikation dieser hämodyna...
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Pulmonary vascular disease represents one of the most frequent complications in systemic sclerosis leading to increased mortality. The recognition and appropriate clinical management of early pulmonary vascular disease could significantly improve the prognosis of affected patients. Early pulmonary vascular disease is characterized by the histologic...
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Rationale Our systematic analysis of anion channels and transporters in idiopathic pulmonary arterial hypertension (IPAH) showed marked upregulation of the Cl ⁻ channel TMEM16A gene. Objective We hypothesised that TMEM16A overexpression might represent a novel vicious circle in the molecular pathways causing PAH. Methods and results We investigat...
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Exercise Doppler echocardiography has been implemented for applications beyond coronary artery disease detection but with a great variability of protocols to assess early-stage pulmonary vascular disease and/or left heart failure. � The Right Heart International Network (RIGHT-NET) is a large prospective clinical and echocardiography observational...
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The diagnosis of idiopathic pulmonary arterial hypertension (iPAH) is complex and, besides invasive hemodynamic evaluation, includes several diagnostic steps to exclude any underlying diseases. The role of a decreased diffusion capacity of the lung for carbon monoxide (DLCO) is a matter of discussion. Here, we present a 76-year-old man with a smoki...
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Objectives of this European Respiratory Society task force were to summarise current studies, to develop strategies for future research and to increase availability and awareness of exercise training for pulmonary hypertension (PH) patients. An evidence-based approach with clinical expertise of the task force members, based on both literature searc...
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Background Rare genetic variants cause pulmonary arterial hypertension, but the contribution of common genetic variation to disease risk and natural history is poorly characterised. We tested for genome-wide association for pulmonary arterial hypertension in large international cohorts and assessed the contribution of associated regions to outcomes...
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Severe pulmonary hypertension (PH) is rare in chronic obstructive pulmonary disease (COPD). Pulmonary arterial hypertension drugs are vasodilators and may cause severe side effects in these patients. Hence, they are not recommended except in right heart failure on an individual basis. Imatinib, a tyrosine-kinase-inhibitor, has no direct vasodilator...
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Pulmonary arterial hypertension (PAH) is a rare disease characterized by increased pulmonary pressure and vascular remodelling as a consequence of smooth muscle cell proliferation, endothelial cell dysfunction and inflammatory infiltrates. Meprin α is a metalloproteinase whose substrates include adhesion and cell–cell contact molecules involved in...
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In the summer of 2016, delegates from the German Society of Cardiology (DGK), the German Respiratory Society (DGP), and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the...
Article
Right heart catheterization (RHC) is the gold standard for the diagnosis and classification of pulmonary hypertension. Significant expertise is required for safely performing a full RHC and for the acquisition of reliable and reproducible information. Physicians performing an RHC should have adequate training not only in vascular access, catheter i...
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The Right Heart International Network is a multicenter international study aiming to prospectively collect exercise Doppler echocardiography tests of the right heart pulmonary circulation unit (RHPCU) in large cohorts of healthy subjects, elite athletes, and individuals at risk of or with overt pulmonary hypertension. It is going to provide standar...
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Full-text available
Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc) associated with high morbidity and mortality. There are several biomarkers of SSc-PAH, reflecting endothelial physiology, inflammation, immune activation, extracellular matrix, metabolic changes, or cardiac involvement. Biomarkers associated with diagnosis, d...
Preprint
Full-text available
Background: Pulmonary arterial hypertension (PAH) is a rare disorder leading to premature death. Rare genetic variants contribute to disease etiology but the contribution of common genetic variation to disease risk and outcome remains poorly characterized. Methods: We performed two separate genome-wide association studies of PAH using data across 1...
Article
Although often clinically unrecognized, the pulmonary circulation is frequently affected in patients with chronic obstructive pulmonary disease (COPD), and the presence of pulmonary vascular disease is associated with poor prognosis and frequent exacerbations. In most cases the presenting pulmonary hypertension (PH) is relatively mild, but in a sub...
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Full-text available
Pulmonary arterial hypertension (PAH) is a rare disorder with a poor prognosis. Deleterious variation within components of the transforming growth factor-β pathway, particularly the bone morphogenetic protein type 2 receptor (BMPR2), underlies most heritable forms of PAH. To identify the missing heritability we perform whole-genome sequencing in 10...