Benjamin Egenlauf

Benjamin Egenlauf
Universität Heidelberg · Thorax Clinic - Center for Pulmonary Hypertension

MD

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86
Publications
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601
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Publications

Publications (86)
Article
Aims To systematically assess late outcomes of acute pulmonary embolism (PE) and to investigate the clinical implications of post-PE impairment (PPEI) fulfilling prospectively defined criteria. Methods and results A prospective multicentre observational cohort study was conducted in 17 large-volume centres across Germany. Adult consecutive patient...
Article
Full-text available
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 rel...
Article
Full-text available
Background A genetic predisposition can lead to the rare disease pulmonary arterial hypertension (PAH). Most mutations have been identified in the gene BMPR2 in heritable PAH. However, as of today 15 further PAH genes have been described. The exact prevalence across these genes particularly in other PAH forms remains uncertain. We present the distr...
Article
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 h...
Article
BACKGROUND: 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. OBJECTIVE: To assess the utility of CTPA-based cardiac chamber volumetric measurements for diagnosis of PH in comparison with echoc...
Article
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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. Met...
Preprint
Full-text available
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. Met...
Article
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) i...
Article
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 superv...
Article
Full-text available
Background: The objective of this study was to analyze prognostic factors and risk stratification in patients with pulmonary arterial hypertension (PAH) and comorbidities. Methods: Patients with invasively diagnosed PAH were included in the analysis. Comorbidities were clinically diagnosed as proposed in the 6th World Symposium of pulmonary hype...
Article
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Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease which is often caused by recurrent emboli. These are also frequently found in patients with myeloproliferative diseases. While myeloproliferative diseases can be caused by gene defects, the genetic predisposition to CTEPH is largely unexplored. Therefore, the objective of this...
Article
Background In this study, we investigated the impact of the new haemodynamic definition of pulmonary arterial hypertension (PAH) as proposed by the 6th PH World Symposium on phenotypes and survival in patients with systemic sclerosis (SSc). Methods In SSc patients who were prospectively and consecutively screened for PAH including right heart cath...
Article
Full-text available
Objective: The objective of this randomized, placebo-controlled, double-blind, parallel group, trial was to assess the effect of ambrisentan on mean pulmonary arterial pressure (mPAP) in patients with systemic sclerosis (SSc) and mildly elevated pulmonary hypertension (PH). Methods: Thirty-eight SSc patients with mildly elevated mPAP at rest bet...
Article
Background Patients with systemic sclerosis (SSc) are at high risk of developing concomitant pulmonary hypertension (PH) which has a crucial impact on the patients' symptoms, quality of life and prognosis. Purpose The aim of this study was to analyse the impact of the new hemodynamic definition of precapillary PH as proposed at the 2018 World Symp...
Article
Objectives To assess the diagnostic accuracy of automated 3D volumetry of central pulmonary arteries using computed tomography pulmonary angiography (CTPA) for suspected pulmonary hypertension alone and in combination with echocardiography. Methods This retrospective diagnostic accuracy study included 70 patients (mean age 66.7, 48 female) assesse...
Article
GATA2 deficiency is characterized by monocytopenia, deficiency of dendritic cells, and a variable degree of lymphocytopenia affecting B cells and NK cells, leading to an enhanced risk of mycobacterial, viral, and fungal infections. Here we present a patient with a heterozygous intronic GATA2 mutation who acquired a fatal disseminated mycosis due to...
Article
Full-text available
Objective The objective of this prospective study was to evaluate right ventricular function and pulmonary arterial compliance (PAC=stroke volume/pulse pressure) at rest and during exercise in patients with systemic sclerosis (SSc) with normal mean pulmonary artery pressures (mPAP) at rest, mildly elevated mPAP (mPAP 21‐24mmHg) and manifest pulmona...
Article
Full-text available
Background Riociguat is a soluble guanylate cyclase stimulator approved for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTPEH). The objective of this study was to evaluate right heart size and function assessed by echocardiography during long term treatment with riociguat. Methods Patients who started r...
Article
Full-text available
Background The objective of this study was to assess, whether right atrial (RA) and ventricular (RV) size is related to RV pump function at rest and during exercise in patients with pulmonary arterial hypertension (PAH). Methods We included 54 patients with invasively diagnosed PAH that had been stable on targeted medication. All patients underwen...
Article
In June 2016, members of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) met for a Consensus Conference in Cologne, Germany. Aim of this Conference was to compile consensus based practice recommendations based on the 2015 European Pulmonary Hypertension g...
Article
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Background: Right ventricular (RV) dysfunction is a major prognostic predictor in pulmonary arterial hypertension (PAH). Objectives: The objective of this study was to assess the prognostic impact of a newly developed index merging haemodynamic parameters into 1 variable. Methods: We retrospectively assessed 2 cohorts of 248 patients (164 from...
Conference Paper
Background In patients with systemic sclerosis (SSc) borderline mean pulmonary arterial pressures (mPAP; 21–24 mmHg at rest) are a frequent finding and could represent an intermediate stage between normal pulmonary pressures and manifest pulmonary hypertension (PH).¹. Objectives The objective of this prospective study was to compare right ventricu...
Article
Full-text available
Background: Despite optimized medical therapy, severe idiopathic pulmonary arterial hypertension (IPAH) is a devastating disease with a poor outcome. Autoantibodies have been detected in IPAH that can contribute to worsening of the disease. Objectives: The objective of this prospective, open-label, single-arm, multicenter trial was to evaluate t...
Article
Background: Combination therapy with the phosphodiesterase type 5 inhibitors (PDE-5i) sildenafil or tadalafil and the endothelin receptor antagonists (ERA) bosentan, ambrisentan, or macitentan may cause mutual pharmacokinetic interactions in patients with pulmonary arterial hypertension (PAH). Objective: The objective of this study was to analyz...
Article
Background: Subcutaneous treprostinil has dose-dependent beneficial effects in patients with severe pulmonary arterial hypertension, but adverse effects like infusion site pain can lead to treatment discontinuation. Objectives: The objective of this study was to evaluate safety, tolerability and clinical effects of a rapid up-titration dosing re...
Article
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-s...
Article
Full-text available
Introduction: Aim of this study was to compare plasma concentrations in PAH patients according to different combination treatments and to identify combinations that cause relevant interactions. Methods: PAH patients receiving a stable combination treatment with Endothelin-Receptor-Antagonists (ERA) and Phosphodiesterase-type-5 inhibitors (PDE-5I) f...
Article
Aims: This prospective randomized controlled study investigates the effects of exercise training on peak VO2/kg, hemodynamics and further clinically relevant parameters in pulmonary hypertension (PH) patients. Methods: Eighty-seven patients with pulmonary arterial hypertension and inoperable chronic thromboembolic PH (54% female, 56±15years, 84% WH...
Article
Full-text available
Introduction: Combination of the phosphodiesterase-type 5 (PDE-5) inhibitor sildenafil (SIL) and the endothelin receptor antagonist (ERA) bosentan (BOS) may cause drug interactions in patients with pulmonary arterial hypertension (PAH). The objective of this study was to analyse drug plasma concentrations in PAH-patients during BOS-SIL combination...
Article
Full-text available
Introduction: Combination therapy of the phosphodiesterase-type 5 (PDE-5) inhibitor sildenafil and the endothelin receptor antagonist (ERA) bosentan may cause drug interactions in patients with pulmonary arterial hypertension (PAH). The objective of this study was to analyse plasma drug concentrations in PAH-patients during bosentan-sildenafil comb...
Article
Full-text available
Introduction: Combination therapy of the phosphodiesterase-type 5 inhibitors (PDE-5I) sildenafil (SIL) or tadalafil (TAD) and endothelin receptor antagonists (ERA) bosentan (BOS), ambrisentan (AMB), or macitentan (MAC) may cause mutual interactions in patients with pulmonary arterial hypertension (PAH). Aim of this study was to compare plasma conce...
Article
Full-text available
Background: The objective of this open label, multi-centre study was to evaluate safety, tolerability and clinical effects of a rapid up-titration dosing regimen of subcutaneous (SC) treprostinil using pro-active infusion site pain management in patients with severe pulmonary arterial hypertension (PAH). Methods: Safety and tolerability were assess...
Article
Full-text available
Riociguat is a soluble guanylate cyclase stimulator approved for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). The objective of this study was to evaluate the change of right heart size and function assessed by echocardiography during long-term treatment with riociguat. We assessed patients who sta...
Article
Full-text available
Background: Riociguat is a soluble guanylate cyclase stimulator approved for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). The objective of this study was to evaluate the change of right heart size and function assessed by echocardiography during long-term treatment with riociguat. Methods: We asse...
Article
Full-text available
Background: The objective of this prospective, 16-week, open-label, multi-centre trial was to evaluate the safety/tolerability of rapid dose-titration of subcutaneous (SC) treprostinil in patients with PAH. Methods: SC treprostinil therapy was initiated at 2 ng/kg/min and then up-titrated in 1-2 ng/kg/min increments every 12 hours (whilst in hospit...
Article
Full-text available
The impact of exercise training on the right heart and pulmonary circulation has not yet been invasively assessed in patients with pulmonary hypertension (PH) and right heart failure. This prospective randomized controlled study investigates the effects of exercise training on peak VO2/kg, haemodynamics, and further clinically relevant parameters i...
Article
Full-text available
In patients with systemic sclerosis (SSc) associated pulmonary arterial hypertension (SSc-APAH) is the leading cause for death. The objective of this prospective screening study was to analyse sensitivity and specificity of stress-Doppler echocardiography (SDE) in detecting pulmonary hypertension (PH). Pulmonary artery pressures and further paramet...
Article
Full-text available
The right ventricular (RV) area is important for diagnosis and follow-up in patients with various diseases, such as in pulmonary hypertension. The aim of this study was to define the reference ranges of the end-diastolic RV area in healthy adults and to assess the determining factors. In the first part of the study 860 healthy subjects (37.6% femal...