catheter-based-endovascular-angioplasty-for-fibrosing-mediastinitis

Catheter-based Endovascular Angioplasty for Fibrosing Mediastinitis-associated Pulmonary Vein Stenosis

Fibrosing mediastinitis (FM) is a rare but severe disease characterized by abnormal proliferation of fibrous tissue within the mediastinum. Pulmonary vein ...

Gansu Provincial Hospital

Fibrosing mediastinitis (FM) is a very rare disease, often caused by histoplasmosis capsulatum, tuberculosis, sarcoidosis, autoimmunity and other diseases, such as IgG 4-related diseases. Fibrous structures in the mediastinum compress the pulmonary artery, pulmonary vein, superior vena cava, esophagus, trachea and cardiac vessels, leading to clinical symptoms. Drug therapeutic modality for pulmonary vein stenosis (PVS) caused by FM is palliative in essence and with limited efficacy, whereas surgical treatment causes high mortality. In recent years, catheter-based treatment to FM-caused PVS has emerged as a promising therapeutic modality, however, the safety and effectiveness of this modality remain unclear. Therefore, a systematic review on the safety and efficacy of the catheter-based treatment for PVS caused by FM was performed, in the hope to shed lights on the alternative therapeutic strategy to this fatal disease.

Balloon angioplasty or stent implantation for pulmonary vein stenosis caused by fibrosing mediastinitis: a systematic review.

 Acute right heart failure (RHF) is the main cause of death in patients with acute pulmonary embolism and emergent pulmonary hypertension. However, the molecular mechanisms underpinning the acute RHF and the interactions between the right (RV) and left ventricles (LVs) under the diseased condition remain unknown.  The Sprague Dawley male rats were randomly divided into the normal control, sham, and pulmonary artery banding (PAB) groups. One hour after the PAB operation, after measuring the haemodynamic and anatomical parameters, the free walls of RV and LV were harvested to detect the differential gene expression profiling by high-throughput RNA sequencing. The results showed that the PAB lead to 50-60% obstruction of the main pulmonary artery, which was accompanied by the significant elevation in the positive rate of rise in RV pressure and the maximum RV pressure as compared to the sham group. Moreover, compared with the counterparts in the sham group, the RV and LV in the PAB group exhibited 2057 differentially expressed genes (DEGs, 1159 upregulated and 898 downregulated) and 1196 DEGs (709 upregulated and 487 downregulated), respectively (DEG criteria: |log fold change| ≥1,  value ≤0.05). In comparison to the sham group, the enriched pathways in the PAB group include nuclear factor-κB signalling pathway, extracellular matrix-receptor interaction, and nucleotide oligomerization domain-like receptor signalling pathway.  The PAB rat model exhibited the haemodynamic and gene expression changes in the RV that lead to acute RHF. Further, the acute RHF induced by pressure overload also caused gene expression changes in the LV, suggesting the molecular interactions between the RV and LV under the diseased condition.

RNA-sequencing analysis of gene expression in a rat model of acute right heart failure.

Right ventricular function (RVF) is an independent predictor of prognosis for patients undergoing aortic valve replacement: transcatheter aortic valve replacement (TAVR) or surgical aortic valve replacement (SAVR). The effect of transfemoral aortic valve replacement (TF-TAVR) on RVF is uncertain. We aimed to perform a meta-analysis of the effect of TF-TAVR on RVF in patients with aortic stenosis (AS) and compare the effect of TF-TAVR with SAVR.

Meta-analysis of right ventricular function in patients with aortic stenosis after transfemoral aortic valve replacement or surgical aortic valve replacement.

Rescue of pulmonary artery intra-stent re-stenosis by unzipping an under-sized stent in an adult patient with fibrosing mediastinitis.

Stenting for pulmonary artery stenosis resistant to balloon pulmonary angioplasty in chronic thrombo-embolic pulmonary hypertension: a bail out strategy.

A Whodunit on a Disappeared Pulmonary Artery.

Pulmonary Vein Stenosis With Normal Pulmonary Artery Wedge Pressure.

Pulmonary hypertension (PH) is a progressive and severe disorder in pulmonary hemodynamics. PH can be fatal if not well managed. Fibrosing mediastinitis (FM) is a rare and benign fibroproliferative disease in the mediastinum, which may lead to pulmonary vessel compression and PH. PH caused by FM (PH-FM) is a pathologic condition belonging to group 5 in the World Health Organization PH classification. PH-FM has a poor prognosis because of a lack of effective therapeutic modalities and inappropriate diagnosis. With the development of percutaneous pulmonary vascular interventional therapy, the prognosis of PH-FM has been greatly improved in recent years. This article provides a comprehensive review on the epidemiology, pathophysiologic characteristics, clinical manifestations, diagnostic approaches, and treatment modalities of PH-FM based on data from published reports and our medical center with the goal of facilitating the diagnosis and treatment of this fatal disease.

Pulmonary Hypertension Caused by Fibrosing Mediastinitis.

This study aims to develop a scoring system for evaluating the degree of pulmonary vascular stenosis in fibrosing mediastinitis (FM).

Pulmonary vascular stenosis scoring in fibrosing mediastinitis.

Acute coronary syndrome (ACS) is a severe type of coronary heart disease (CHD) with increasing prevalence and significant challenges for prevention and treatment. Metabolomics is an emerging technology with intrinsic dynamics and flexibility to better delineate the phenotypic and metabolic alterations in organisms at the time of altered pathological states. It provides new insights into the complex pathological mechanisms of cardiovascular disease and contributes to the early detection, monitoring and evaluation of ACS. In this review, we analyze and summarize the literature related to ACS metabolomics which has contributed to the diagnosis and prevention of ACS.

Progress in the Metabolomics of Acute Coronary Syndrome.

Advances in cancer treatment have increased patient survival rates, shifting clinical focus towards minimizing treatment-related morbidity, including cardiovascular issues. Since echocardiography allows for a comprehensive non-invasive assessment at all cancer stages, it is well suited to monitor cardiovascular disease secondary to oncology treatment. This has earned it significant attention in the study of cardiac tumors and treatment-induced cardiac alterations. Ultrasound methods-ranging from transthoracic and transesophageal echocardiography to ultrasound diagnostic techniques including myocardial strain imaging, myocardial work indices, three-dimensional cardiac imaging-offer a holistic view of both the tumor and its treatment impact cardiac function. Stress echocardiography, myocardial contrast echocardiography, and myocardial acoustic angiography further augment this capability. Together, these echocardiographic techniques provide clinicians with early detection opportunities for cardiac damage, enabling timely interventions. As such, echocardiography continues to be instrumental in monitoring and managing the cardiovascular health of oncology patients, complementing efforts to optimize their overall treatment and survival outcomes.

Progress in the Use of Echocardiography in Patients with Tumors.

Fibrosing mediastinitis (FM) is a rare yet fatal condition, caused by different triggers and frequently culminating in the obstruction of the pulmonary vasculature and airways, often leading to pulmonary hypertension and right heart failure. Percutaneous transluminal pulmonary venoplasty (PTPV) is an emerging treatment for pulmonary vein stenosis (PVS) caused by FM. Our previous study showed as high as 24% of in-stent restenosis (ISR) in FM. However, the predictors of ISR are elusive.

Incidence and predictors of in-stent restenosis following intervention for pulmonary vein stenosis due to fibrosing mediastinitis.

Fibrosing mediastinitis (FM) is a rare and benign disease affecting the mediastinum and often causes pulmonary hypertension (PH). Timely diagnosis of PH caused by FM is clinically important to mitigate complications such as right heart failure in affected individuals. This retrospective study aimed to analyze the CT imaging characteristics of tuberculosis (TB) related FM in patients with (TB). Additionally, the study investigates the underlying reasons contributing to the manifestation of symptoms.

Image Findings from Dual-phase Computed Tomography Pulmonary Angiography for Diagnosing Tuberculosis-associated Fibrosing Mediastinitis.

The 6-min walk test (6MWT) has significant prognostic value, but requires long walking distances and lacks evaluation of exercise speed. This study aimed to investigate the clinical utility of a new walk test, the 18-meter walk test (18MWT), in patients with pulmonary arterial hypertension (PAH) as a complement to the 6MWT. In summary, a total of 117 patients with PAH from January 2018 to December 2022 were included. Spearman correlation, Cox regression, and Kaplan-Meier analysis were utilized to demonstrate the value of 18MWT in predicting disease severity and clinical worsening. The median time to complete the 18MWT was 12.8 s (interquartile range: 11.3-14.6 s). 18MWT completion time showed significant correlations with indicators such as N-terminal pro-brain natriuretic peptide and 6MWT distance. Adjusted Cox regression showed 18MWT time remained an independent predictor of clinical worsening (hazard ratio = 1.10; 95% confidence interval: 1.01-1.21;  = 0.026). A simplified risk stratification using WHO functional class, 6MWT distance, 18MWT time and NT-proBNP was predictive of 1-year clinical outcome. These results suggest that the 18MWT provides clinicians with an efficient measure that can be used to evaluate the disease severity of PAH patients and to identify those patients at greater risk for future clinical worsening as a complement to the 6MWT.

Article	Year
Balloon angioplasty or stent implantation for pulmonary vein stenosis caused by fibrosing mediastinitis: a systematic review. Cardiovascular diagnosis and therapy\| PubMed ID: 31737523	2019
RNA-sequencing analysis of gene expression in a rat model of acute right heart failure. Pulmonary circulation\| PubMed ID: 32128157	2020
Meta-analysis of right ventricular function in patients with aortic stenosis after transfemoral aortic valve replacement or surgical aortic valve replacement. Therapeutic advances in chronic disease\| PubMed ID: 32670537	2020
Rescue of pulmonary artery intra-stent re-stenosis by unzipping an under-sized stent in an adult patient with fibrosing mediastinitis. Chinese medical journal\| PubMed ID: 34074843	2021
Stenting for pulmonary artery stenosis resistant to balloon pulmonary angioplasty in chronic thrombo-embolic pulmonary hypertension: a bail out strategy. European heart journal. Case reports\| PubMed ID: 34113761	2021
A Whodunit on a Disappeared Pulmonary Artery. JACC. Cardiovascular interventions\| PubMed ID: 34774473	2021
Pulmonary Vein Stenosis With Normal Pulmonary Artery Wedge Pressure. JACC. Cardiovascular interventions\| PubMed ID: 35033473	2022
Pulmonary Hypertension Caused by Fibrosing Mediastinitis. JACC. Asia\| PubMed ID: 36338410	2022
Pulmonary vascular stenosis scoring in fibrosing mediastinitis. European heart journal. Imaging methods and practice\| PubMed ID: 39045195	2024
Progress in the Metabolomics of Acute Coronary Syndrome. Reviews in cardiovascular medicine\| PubMed ID: 39077017	2023
Progress in the Use of Echocardiography in Patients with Tumors. Reviews in cardiovascular medicine\| PubMed ID: 39077667	2024
Incidence and predictors of in-stent restenosis following intervention for pulmonary vein stenosis due to fibrosing mediastinitis. Orphanet journal of rare diseases\| PubMed ID: 39397011	2024
Image Findings from Dual-phase Computed Tomography Pulmonary Angiography for Diagnosing Tuberculosis-associated Fibrosing Mediastinitis. Current medical imaging\| PubMed ID: 39757667	2025
Role of a New Walk Test in Pulmonary Arterial Hypertension: A Retrospective Cohort Study. Pulmonary circulation\| PubMed ID: 40291436	2025
Safety and Efficacy of Half-Half Inflation Strategy in Pulmonary Artery Stenosis Caused by Fibrosing Mediastinitis. JACC. Asia\| PubMed ID: 40366323	2025

Kaiyu Jiang

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