Pulmonary Hypertension Caused by Fibrosing Mediastinitis

Wang, Aqian; Su, Hongling; Duan, Yichao; Liu, Yu; Deng, Minghua; Long, Xiaozhou; Wang, Haijun; Zhang, Min; Zhang, Yan; Cao, Yunshan

doi:10.1016/j.jacasi.2021.11.016

Cited by 27 publications

(40 citation statements)

References 86 publications

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“…[31,41,55,60,70,80] Specifically, FM dyad (PH signs and pulmonary atelectasis), FM triad (FM dyad plus refractory pleural effusion or upper lobe pulmonary congestion/ interstitial pulmonary edema), and FM tetralogy (FM dyad plus pleural effusion and upper lobe interstitial pulmonary edema) have been used as important cues for FM-PVS. [63] Additionally, clinicians should suspect PVI-PVS in new-onset PH after PVI.…”

Section: Clinical Presentation Of Pvsmentioning

confidence: 99%

“…[33,61,62] Patients usually have a history of Histoplasma capsulatum or Mycobacterium tuberculosis infection. [63] The hyperplasia of fibrous tissue enfolds, infiltrates, and compresses adjacent mediastinal structures, resulting in stenosis of pulmonary vessels, bronchus, superior vena cava, and esophagus. [64,65] The common presenting complaints include dyspnea, cough, recurrent pulmonary infections, hemoptysis, and chest pain.…”

Section: Etiology Classification Pathophysiology and Clinical Phenotypementioning

confidence: 99%

“…[93] Typical FM dyad/triad/tetralogy is also observed in patients with FM-PVS. [63] CTPV is often used as a routine preoperative evaluation and has important clinical significance for the diagnosis and guidance of treatment [Figure 4]. Notably, previous studies have reported that CT may grossly overestimate PV occlusion (compared to pulmonary angiography).…”

Section: Imaging Of Pvsmentioning

confidence: 99%

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Pulmonary Vein Stenosis: A Review

Jia

Wang

et al. 2023

Cardiology Discovery

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Pulmonary vein stenosis (PVS) is an extremely rare and lethal disease caused by multiple etiologies. PVS has a bimodal distribution in the population, affecting children and adults. Congenital PVS is the usual PVS type in children, which sometimes develops after cardiothoracic surgery. Acquired PVS, in turn, is the most common PVS type in adults. A review of the relevant literature has shown that PVS after radiofrequency ablation of atrial fibrillation is the most common, as well as that caused by compression of proliferative fibrous tissues or tumor in the mediastinum (eg, PVS caused by fibrosing mediastinitis, lung tumors, metastases, etc). This article provides a comprehensive review of PVS in terms of embryology and anatomy, etiology and triggers, classification, clinical symptoms and signs, treatment, and prognosis, intending to promote the understanding and treatment of this disease.

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Section: Clinical Presentation Of Pvsmentioning

confidence: 99%

Section: Etiology Classification Pathophysiology and Clinical Phenotypementioning

confidence: 99%

Section: Imaging Of Pvsmentioning

confidence: 99%

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Pulmonary Vein Stenosis: A Review

Jia

Wang

et al. 2023

Cardiology Discovery

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“…Pulmonary hypertension caused by fibrosing mediastinitis (PH-FM), belonging to group 5 of the World Health Organization PH classification, is a rare disease that eventually leads to right heart failure (RHF) and death. 1 , 2 The 5-year mortality rate of this disease can be as high as 46% if with no proper treatment. 3 However, misdiagnosis and underdiagnosis of PH-FM are common due to the rarity, lack of awareness, and non-specificity of symptoms of this fatal disease.…”

Section: Introductionmentioning

confidence: 99%

“…10 Previous study indicated that CXR features, including FM dyad (prominent main pulmonary artery and atelectasis) and FM triad (the dyad plus refractory pleural effusion), are important diagnostic clues for PH-FM. 2 However, the accuracy of CXR features for diagnosis of PH-FM remains unknown. Therefore, this study sought to systematically summarize the CXR features of the patients with PH-FM, and to evaluate the accuracy of CXR for PH-FM diagnosis, in the hope to improve the early detection and diagnosis of PH-FM among PH patients.…”

Section: Introductionmentioning

confidence: 99%

Chest X-ray features facilitate screening for pulmonary hypertension caused by fibrosing mediastinitis

Zhou

Chen

et al. 2022

Therapeutic Advances in Chronic Disease

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Background: Misdiagnosis and underdiagnosis of pulmonary hypertension caused by fibrosing mediastinitis (PH-FM) are considerably prevalent due to unspecific symptoms and as well as the lack of awareness of this fatal disease. Objectives: The aim of this study was to evaluate the diagnostic accuracy of the chest X-ray (CXR) for screening the patients with PH-FM from those with pulmonary hypertension (PH). Design: This was a retrospective observational cohort study. Methods: The patients with suspected PH were recruited between October 2014 and October 2020. All the clinical data and CXR findings were collected. The sensitivity, specificity, and likelihood ratio of the CXR features were calculated. Logistic regression was used to identify the factors associated with the CXR characteristics and FM and to generate a prediction model. Finally, the diagnostic efficiency of the prediction model was evaluated using nomogram and internal validation. Results: The patients with PH-FM ( n = 36) and PH caused by the diseases other than FM (PH-non-FM, n = 62) were enrolled. The CXR features, including atelectasis, pleural effusion, consolidation, nodules, calcification, interlobular septal thickening, and interstitial reticulation, were more prevalent in patients with PH-FM than in those with PH-non-FM (all p < 0.05). Atelectasis had a specificity of 97%, a sensitivity of 50%, and a greater accuracy for diagnosing of PH-FM [area under the curve (AUC) = 0.720; 95% CI: 0.634–0.806] than the other factors did. The combination of tuberculosis, natural logarithmic NT-proBNP (lnBNP), atelectasis, pleural effusion, and prominent right heart border constituted a prediction model to distinguish the PH-FM from the PH-non-FM, with a sensitivity of 91.7% and a specificity of 83.9%. The model demonstrated good prediction performance by showing an AUC of 0.922 (95% CI: 0.861–0.983) in the internal validation. Conclusion: In this study, atelectasis was the most specific and accurate CXR characteristic for identifying PH-FM in the PH patients. The combination of atelectasis, pleural effusion, prominent right heart border, tuberculosis, and lnBNP constituted a prediction model that distinguished the PH-FM patients from the PH-non-FM ones with good performance.

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