Joanna Pepke‐Żaba
A5002804694- Pulmonary Hypertension Research and Treatments
- Cardiovascular Issues in Pregnancy
- Venous Thromboembolism Diagnosis and Management
- Vascular Anomalies and Treatments
- Cardiovascular Function and Risk Factors
- Heart Failure Treatment and Management
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Liver Disease and Transplantation
- Congenital Heart Disease Studies
- Nitric Oxide and Endothelin Effects
- Medical Imaging and Pathology Studies
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Cardiac Arrhythmias and Treatments
- Cardiovascular Effects of Exercise
- Cardiac Valve Diseases and Treatments
- Atrial Fibrillation Management and Outcomes
- Cardiac tumors and thrombi
- Hemodynamic Monitoring and Therapy
- Liver Disease Diagnosis and Treatment
- RNA modifications and cancer
- Respiratory Support and Mechanisms
- Acute Ischemic Stroke Management
- Neonatal Respiratory Health Research
- Cardiac, Anesthesia and Surgical Outcomes
- Bone health and treatments
Papworth Hospital
2016-2025
University of Cambridge
2009-2025
Papworth Hospital NHS Foundation Trust
2015-2024
Council of Science Editors
2020
Addenbrooke's Hospital
1990-2019
St Vincent's Hospital Sydney
2018
Medical University of Vienna
2011-2016
St. Antonius Ziekenhuis
2016
KU Leuven
2016
MRC Biostatistics Unit
2016
2022 ESC/ERS pulmonary hypertension guidelines incorporate changes and adaptations focusing on clinical management https://bit.ly/3QtUvb4
The Task Force has classified and ranked the usefulness or efficacy of recommended procedure and/or treatments Level Evidence as indicated in tables below: Classes RecommendationsClass I general agreement that a given diagnostic procedure/treatment is beneficial, useful effective; Class II Conflicting evidence divergence opinion about usefulness/efficacy treatment; IIa Weight evidence/opinion favour usefulness/efficacy; IIb Usefulness/efficacy less well established by evidence/opinion; III...
Background— Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by endarterectomy. The clinical characteristics and current management patients enrolled in an international CTEPH registry was investigated. Methods Results— included 679 newly diagnosed (≤6 months) consecutive CTEPH, from February 2007 until January 2009. Diagnosis confirmed right heart catheterization,...
Background— Inflammation is a feature of pulmonary arterial hypertension (PAH), and increased circulating levels cytokines are reported in patients with PAH. However, to date, no information exists on the significance elevated or their potential as biomarkers. We sought determine range PAH examine impact survival relationship hemodynamic indexes. Methods Results— measured serum (tumor necrosis factor-α, interferon-γ interleukin-1β, -2, -4, -5, -6, -8, -10, -12p70, -13) using ELISAs...
Rationale: Pulmonary arterial hypertension in association with connective tissue disease (CTD-PAH) has historically had a poor prognosis, 1-year survival rate among patients systemic sclerosis–associated pulmonary (SSc-PAH) of 45%. However, more therapies have become available.Objectives: To investigate the and characteristics all diagnosed CTD-PAH U.K. service.Methods: National registry incident cases consecutively between January 2001 June 2006.Measurements Main Results: Patients (429; 73%...
Background— Chronic thromboembolic pulmonary hypertension, a rare complication of acute embolism, is characterized by fibrothrombotic obstructions large arteries combined with small-vessel arteriopathy. It can be cured endarterectomy, and clinically improved medical therapy in inoperable patients. A European registry was set up 27 centers to evaluate long-term outcome correlates 2 distinct populations operated not-operated patients who have chronic hypertension. Methods Results— total 679...
Endothelial cells release endothelium-derived relaxing factor (EDRF) in a variety of vascular beds, including the pulmonary circulation. However, role EDRF-mediated pulmonary-artery relaxation chronic hypoxic lung disease is unknown.
Incident pulmonary arterial hypertension was underrepresented in most registries and may have a different disease profile to prevalent disease.To determine the characteristics outcome of purely incident, treatment-naive cohort idiopathic, heritable, anorexigen-associated changes presentations survival over past decade United Kingdom Ireland.All consecutive newly diagnosed patients from 2001 2009 were identified prospectively.A total 482 (93% 5% 2% hypertension) diagnosed, giving rise an...
The management of chronic thromboembolic pulmonary hypertension (CTEPH) has changed over recent years with the growth endarterectomy surgery and availability disease-modifying therapies.To investigate prognosis CTEPH in a national setting during years.All incident cases diagnosed one five centers United Kingdom between January 2001 June 2006 were identified prospectively. Information regarding baseline characteristics, treatment, follow-up was subsequently collected from hospital records.A...
Background— For almost 30 years, anticoagulation has been recommended for patients with idiopathic pulmonary arterial hypertension (IPAH). Supporting evidence, however, is limited, and it unclear whether this recommendation still justified in the modern management era should be extended to other forms of (PAH). Methods Results— We analyzed data from Comparative, Prospective Registry Newly Initiated Therapies Pulmonary Hypertension (COMPERA), an ongoing European registry. Survival rates IPAH...
Background— Chronic thromboembolic pulmonary hypertension results from incomplete resolution of emboli. Pulmonary endarterectomy (PEA) is potentially curative, but residual following surgery common and its impact on long-term outcome poorly understood. We wanted to identify factors correlated with poor after specifically define clinically relevant post-PEA. Methods Results— Eight hundred eighty consecutive patients (mean age, 57 years) underwent PEA for chronic hypertension. Patients...
Pulmonary arterial hypertension (PAH) is a rare disorder with poor prognosis. Deleterious variation within components of the transforming growth factor-β pathway, particularly bone morphogenetic protein type 2 receptor (BMPR2), underlies most heritable forms PAH. To identify missing heritability we perform whole-genome sequencing in 1038 PAH index cases and 6385 PAH-negative control subjects. Case-control analyses reveal significant overrepresentation variants ATP13A3, AQP1 SOX17, provide...
Pulmonary hypertension (PH) is hemodynamically classified as pre-capillary (as seen in idiopathic pulmonary arterial [IPAH]) or post-capillary heart failure with preserved ejection fraction [HFpEF]). Overlaps between these conditions exist. Some patients present risk factors for left disease but PH, whereas HFpEF may have combined pre- and PH. This study sought to further characterize similarities differences among patient populations either PH-HFpEF IPAH. We used registry data analyze...
Chronic thromboembolic disease is characterised by persistent pulmonary occlusions without hypertension. Early surgical treatment with endarterectomy may improve symptoms and prevent progression. We sought to assess the outcome of in symptomatic patients chronic disease. Patients a mean artery pressure <25 mmHg at baseline right heart catheterisation treated between January 2000 July 2013 were identified. reassessed 6 months 1 year following surgery. A total 42 underwent surgery median...
Objective— Inflammation and dysregulated angiogenesis are features of endothelial dysfunction in pulmonary hypertension. Neutrophil extracellular traps (NETs), produced by dying neutrophils, contribute to pathogenesis numerous vascular disorders but their role hypertension has not been studied. We sought evidence (NETs) formation investigated the effect NETs on function. Approach Results— Plasma lung tissues patients with were analyzed for NET markers. The effects function studied vitro...
Background: Pulmonary arterial hypertension (PAH) is a heterogeneous disorder with high mortality. Methods: We conducted comprehensive study of plasma metabolites using ultraperformance liquid chromatography mass spectrometry to identify patients at risk early death, who respond well treatment, and provide novel molecular insights into disease pathogenesis. Results: Fifty-three circulating distinguished well-phenotyped idiopathic or heritable PAH (n=365) from healthy control subjects (n=121)...