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European Heart Journal Aug 2023The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European...
Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: a clinical consensus statement of the ESC working group on pulmonary circulation and right ventricular function.
The current treatment algorithm for chronic thromboembolic pulmonary hypertension (CTEPH) as depicted in the 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) guidelines on the diagnosis and treatment of pulmonary hypertension (PH) includes a multimodal approach of combinations of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and medical therapies to target major vessel pulmonary vascular lesions, and microvasculopathy. Today, BPA of >1700 patients has been reported in the literature from centers in Asia, the US, and also Europe; many more patients have been treated outside literature reports. As BPA becomes part of routine care of patients with CTEPH, benchmarks for safe and effective care delivery become increasingly important. In light of this development, the ESC Working Group on Pulmonary Circulation and Right Ventricular Function has decided to publish a document that helps standardize BPA to meet the need of uniformity in patient selection, procedural planning, technical approach, materials and devices, treatment goals, complications including their management, and patient follow-up, thus complementing the guidelines. Delphi methodology was utilized for statements that were not evidence based. First, an anatomical nomenclature and a description of vascular lesions are provided. Second, treatment goals and definitions of complete BPA are outlined. Third, definitions of complications are presented which may be the basis for a standardized reporting in studies involving BPA. The document is intended to serve as a companion to the official ESC/ERS guidelines.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Embolism; Pulmonary Circulation; Ventricular Function, Right; Angioplasty, Balloon; Pulmonary Artery; Chronic Disease; Cardiology
PubMed: 37470202
DOI: 10.1093/eurheartj/ehad413 -
EuroIntervention : Journal of EuroPCR... Oct 2022There is a growing clinical and scientific interest in catheter-directed therapy (CDT) of acute pulmonary embolism (PE). Currently, CDT should be considered for patients...
Percutaneous treatment options for acute pulmonary embolism: a clinical consensus statement by the ESC Working Group on Pulmonary Circulation and Right Ventricular Function and the European Association of Percutaneous Cardiovascular Interventions.
There is a growing clinical and scientific interest in catheter-directed therapy (CDT) of acute pulmonary embolism (PE). Currently, CDT should be considered for patients with high-risk PE, in whom thrombolysis is contraindicated or has failed. Also, CDT is a treatment option for initially stable patients in whom anticoagulant treatment fails, i.e., those who experience haemodynamic deterioration despite adequately dosed anticoagulation. However, the definition of treatment failure (primary reperfusion therapy or anticoagulation alone) remains an important area of uncertainty. Moreover, several techniques for CDT are available without evidence supporting one over the other, and variation in practice with regard to periprocedural anticoagulation is considerable. The aim of this position paper is to describe the currently available CDT approaches in PE patients and to standardise patient selection, the timing and technique of the procedure itself as well as anticoagulation regimens during CDT. We discuss several clinical scenarios of the clinical evaluation of the "efficacy" of thrombolysis and anticoagulation, including treatment failure with haemodynamic deterioration and treatment failure based on a lack of improvement. This clinical consensus statement serves as a practical guide for CDT, complementary to the formal guidelines.
Topics: Acute Disease; Anticoagulants; Fibrinolytic Agents; Humans; Pulmonary Circulation; Pulmonary Embolism; Thrombolytic Therapy; Treatment Outcome; Ventricular Function, Right
PubMed: 36112184
DOI: 10.4244/EIJ-D-22-00246 -
Thorax Jan 2017Electrical impedance tomography (EIT) has undergone 30 years of development. Functional chest examinations with this technology are considered clinically relevant,... (Review)
Review
Chest electrical impedance tomography examination, data analysis, terminology, clinical use and recommendations: consensus statement of the TRanslational EIT developmeNt stuDy group.
Electrical impedance tomography (EIT) has undergone 30 years of development. Functional chest examinations with this technology are considered clinically relevant, especially for monitoring regional lung ventilation in mechanically ventilated patients and for regional pulmonary function testing in patients with chronic lung diseases. As EIT becomes an established medical technology, it requires consensus examination, nomenclature, data analysis and interpretation schemes. Such consensus is needed to compare, understand and reproduce study findings from and among different research groups, to enable large clinical trials and, ultimately, routine clinical use. Recommendations of how EIT findings can be applied to generate diagnoses and impact clinical decision-making and therapy planning are required. This consensus paper was prepared by an international working group, collaborating on the clinical promotion of EIT called TRanslational EIT developmeNt stuDy group. It addresses the stated needs by providing (1) a new classification of core processes involved in chest EIT examinations and data analysis, (2) focus on clinical applications with structured reviews and outlooks (separately for adult and neonatal/paediatric patients), (3) a structured framework to categorise and understand the relationships among analysis approaches and their clinical roles, (4) consensus, unified terminology with clinical user-friendly definitions and explanations, (5) a review of all major work in thoracic EIT and (6) recommendations for future development (193 pages of online supplements systematically linked with the chief sections of the main document). We expect this information to be useful for clinicians and researchers working with EIT, as well as for industry producers of this technology.
Topics: Adolescent; Adult; Cardiac Output; Child; Child, Preschool; Consensus; Electric Impedance; Humans; Infant; Infant, Newborn; Lung Diseases; Pulmonary Circulation; Respiration, Artificial; Terminology as Topic; Tomography
PubMed: 27596161
DOI: 10.1136/thoraxjnl-2016-208357 -
European Heart Journal Jan 2022This position paper provides a comprehensive guide for optimal follow-up of patients with acute pulmonary embolism (PE), covering multiple relevant aspects of patient...
Optimal follow-up after acute pulmonary embolism: a position paper of the European Society of Cardiology Working Group on Pulmonary Circulation and Right Ventricular Function, in collaboration with the European Society of Cardiology Working Group on Atherosclerosis and Vascular Biology, endorsed by...
This position paper provides a comprehensive guide for optimal follow-up of patients with acute pulmonary embolism (PE), covering multiple relevant aspects of patient counselling. It serves as a practical guide to treating patients with acute PE complementary to the formal 2019 European Society of Cardiology guidelines developed with the European Respiratory Society. We propose a holistic approach considering the whole spectrum of serious adverse events that patients with acute PE may encounter on the short and long run. We underline the relevance of assessment of modifiable risk factors for bleeding, of acquired thrombophilia and limited cancer screening (unprovoked PE) as well as a dedicated surveillance for the potential development of chronic thromboembolic pulmonary hypertension as part of routine practice; routine testing for genetic thrombophilia should be avoided. We advocate the use of outcome measures for functional outcome and quality of life to quantify the impact of the PE diagnosis and identify patients with the post-PE syndrome early. Counselling patients on maintaining a healthy lifestyle mitigates the risk of the post-PE syndrome and improves cardiovascular prognosis. Therefore, we consider it important to discuss when and how to resume sporting activities soon after diagnosing PE. Additional patient-relevant topics that require Focused counselling are travel and birth control.
Topics: Atherosclerosis; Biology; Cardiology; Follow-Up Studies; Humans; Pulmonary Circulation; Pulmonary Embolism; Quality of Life; Ventricular Function, Right
PubMed: 34875048
DOI: 10.1093/eurheartj/ehab816 -
Nature Medicine Feb 2016Although the lung can undergo self-repair after injury, fibrosis in chronically injured or diseased lungs can occur at the expense of regeneration. Here we study how a...
Although the lung can undergo self-repair after injury, fibrosis in chronically injured or diseased lungs can occur at the expense of regeneration. Here we study how a hematopoietic-vascular niche regulates alveolar repair and lung fibrosis. Using intratracheal injection of bleomycin or hydrochloric acid in mice, we show that repetitive lung injury activates pulmonary capillary endothelial cells (PCECs) and perivascular macrophages, impeding alveolar repair and promoting fibrosis. Whereas the chemokine receptor CXCR7, expressed on PCECs, acts to prevent epithelial damage and ameliorate fibrosis after a single round of treatment with bleomycin or hydrochloric acid, repeated injury leads to suppression of CXCR7 expression and recruitment of vascular endothelial growth factor receptor 1 (VEGFR1)-expressing perivascular macrophages. This recruitment stimulates Wnt/β-catenin-dependent persistent upregulation of the Notch ligand Jagged1 (encoded by Jag1) in PCECs, which in turn stimulates exuberant Notch signaling in perivascular fibroblasts and enhances fibrosis. Administration of a CXCR7 agonist or PCEC-targeted Jag1 shRNA after lung injury promotes alveolar repair and reduces fibrosis. Thus, targeting of a maladapted hematopoietic-vascular niche, in which macrophages, PCECs and perivascular fibroblasts interact, may help to develop therapy to spur lung regeneration and alleviate fibrosis.
Topics: Animals; Antibiotics, Antineoplastic; Bleomycin; Calcium-Binding Proteins; Capillaries; Endothelial Cells; Fibroblasts; Fibrosis; Fluorescent Antibody Technique; Humans; Hydrochloric Acid; Intercellular Signaling Peptides and Proteins; Jagged-1 Protein; Lung; Lung Injury; Macrophages; Membrane Proteins; Mice; Oligopeptides; Pulmonary Artery; Pulmonary Circulation; Pulmonary Fibrosis; RNA, Small Interfering; Receptors, CXCR; Receptors, Notch; Regeneration; Serrate-Jagged Proteins; Smad3 Protein; Vascular Endothelial Growth Factor Receptor-1; Wnt Signaling Pathway
PubMed: 26779814
DOI: 10.1038/nm.4035 -
Clinics in Chest Medicine Mar 2021Pulmonary arterial hypertension (PAH) occurs in women more than men whereas survival in men is worse than in women. In recent years, much research has been carried out... (Review)
Review
Pulmonary arterial hypertension (PAH) occurs in women more than men whereas survival in men is worse than in women. In recent years, much research has been carried out to understand these sex differences in PAH. This article discusses clinical and preclinical studies that have investigated the influences of sex, serotonin, obesity, estrogen, estrogen synthesis, and estrogen metabolism on bone morphogenetic protein receptor type II signaling, the pulmonary circulation and right ventricle in both heritable and idiopathic pulmonary hypertension.
Topics: Bone Morphogenetic Protein Receptors, Type II; Estrogens; Female; Heart Ventricles; Humans; Hypertension, Pulmonary; Male; Obesity; Pulmonary Circulation; Serotonin; Sex Characteristics; Signal Transduction
PubMed: 33541615
DOI: 10.1016/j.ccm.2020.10.005 -
Current Topics in Membranes 2018
Topics: Cell Membrane; Endothelium, Vascular; Humans; Lung; Pulmonary Circulation; Vascular Diseases
PubMed: 30360785
DOI: 10.1016/S1063-5823(18)30049-8 -
Paediatric Respiratory Reviews Sep 2022The purpose of this review is to describe the current state of the art in clinical imaging for NICU patients, divided into major areas that correspond to likely... (Review)
Review
The purpose of this review is to describe the current state of the art in clinical imaging for NICU patients, divided into major areas that correspond to likely phenotypes of neonatal respiratory disease: airway abnormalities, parenchymal disease, and pulmonary vascular disease. All common imaging modalities (ultrasound, X-ray, CT, and MRI) are discussed, with an emphasis on modalities that are most relevant to the individual underlying aspects of disease. Some promising aspects of dynamic and functional imaging are included, where there may be future clinical applicability.
Topics: Humans; Infant, Newborn; Bronchopulmonary Dysplasia; Lung; Infant, Newborn, Diseases; Magnetic Resonance Imaging; Pulmonary Circulation; Respiration Disorders
PubMed: 35074281
DOI: 10.1016/j.prrv.2021.12.002 -
Archivos de Cardiologia de Mexico 2022
Topics: Heart; History, Medieval; Pulmonary Circulation
PubMed: 34987236
DOI: 10.24875/ACM.M21000080 -
Journal of the American College of... Aug 2022PVDOMICS (Pulmonary Vascular Disease Phenomics) is a precision medicine initiative to characterize pulmonary vascular disease (PVD) using deep phenotyping. PVDOMICS...
BACKGROUND
PVDOMICS (Pulmonary Vascular Disease Phenomics) is a precision medicine initiative to characterize pulmonary vascular disease (PVD) using deep phenotyping. PVDOMICS tests the hypothesis that integration of clinical metrics with omic measures will enhance understanding of PVD and facilitate an updated PVD classification.
OBJECTIVES
The purpose of this study was to describe clinical characteristics and transplant-free survival in the PVDOMICS cohort.
METHODS
Subjects with World Symposium Pulmonary Hypertension (WSPH) group 1-5 PH, disease comparators with similar underlying diseases and mild or no PH and healthy control subjects enrolled in a cross-sectional study. PH groups, comparators were compared using standard statistical tests including log-rank tests for comparing time to transplant or death.
RESULTS
A total of 1,193 subjects were included. Multiple WSPH groups were identified in 38.9% of PH subjects. Nocturnal desaturation was more frequently observed in groups 1, 3, and 4 PH vs comparators. A total of 50.2% of group 1 PH subjects had ground glass opacities on chest computed tomography. Diffusing capacity for carbon monoxide was significantly lower in groups 1-3 PH than their respective comparators. Right atrial volume index was higher in WSPH groups 1-4 than comparators. A total of 110 participants had a mean pulmonary artery pressure of 21-24 mm Hg. Transplant-free survival was poorest in group 3 PH.
CONCLUSIONS
PVDOMICS enrolled subjects across the spectrum of PVD, including mild and mixed etiology PH. Novel findings include low diffusing capacity for carbon monoxide and enlarged right atrial volume index as shared features of groups 1-3 and 1-4 PH, respectively; unexpected, frequent presence of ground glass opacities on computed tomography; and sleep alterations in group 1 PH, and poorest survival in group 3 PH. PVDOMICS will facilitate a new understanding of PVD and refine the current PVD classification. (Pulmonary Vascular Disease Phenomics Program PVDOMICS [PVDOMICS]; NCT02980887).
Topics: Carbon Monoxide; Cross-Sectional Studies; Humans; Hypertension, Pulmonary; Pulmonary Circulation; Vascular Diseases
PubMed: 35953136
DOI: 10.1016/j.jacc.2022.05.038