A pressing matter—mediastinal fibrosis with near obliteration of the pulmonary arteries

Murphy, Jimmy; Johnston, Nicola; Spence, Mark S.

doi:10.1002/ccd.24561

Cited by 4 publications

(5 citation statements)

References 9 publications

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“…[ 9 ] One of the complications of stenting of the pulmonary arteries with near obliteration is postoperative reperfusion injury as shown in a previous case report. [ 6 ] In our case, with total obstruction of the left pulmonary artery and three pulmonary veins, stenting and surgical repair were not feasible.…”

Section: Discussionmentioning

confidence: 83%

“…[ 3 4 5 ] Involvement of pulmonary vasculature secondary to FM is rare. [ 5 6 ] Treatment of FM is largely empirical and includes pharmacotherapy and percutaneous surgical intervention. [ 4 5 6 ] Here, we report a case of a young woman with severe idiopathic FM who was successfully treated with pulsed steroid therapy followed by maintenance oral steroids and methotrexate with very good long-term disease control.…”

mentioning

confidence: 99%

“…[ 5 6 ] Treatment of FM is largely empirical and includes pharmacotherapy and percutaneous surgical intervention. [ 4 5 6 ] Here, we report a case of a young woman with severe idiopathic FM who was successfully treated with pulsed steroid therapy followed by maintenance oral steroids and methotrexate with very good long-term disease control. To our knowledge, this is the first reported case of FM treated successfully with pulsed steroid therapy and methotrexate.…”

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confidence: 99%

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Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach

2017

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Fibrosing mediastinitis (FM), also known as sclerosing mediastinitis, is an uncommon but serious disease involving the mediastinal structures. A high index of suspicion is essential to establish the diagnosis of FM and starting the appropriate therapy for patients. Here, we report a case of a young female who presented with chest symptoms and subsequently underwent different laboratory and radiologic investigations and an excisional biopsy. The findings of these investigations were consistent with the diagnosis of idiopathic FM. Her disease was associated with complete occlusion of three pulmonary veins and the left main pulmonary artery. The patient was treated with initial high-dose steroids followed by maintenance steroid and methotrexate therapy with very good long-term disease control. Clinical response, high-sensitivity C-reactive protein, and erythrocyte sedimentation rate were used to monitor disease activity and response to therapy.

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Section: Discussionmentioning

confidence: 83%

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confidence: 99%

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confidence: 99%

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Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach

2017

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“…Procedure-related complications associated with endovascular intervention for central vascular obstructions due to FM have been reported to occur in 24% of the cases according to a retrospective review of 40 interventions. The most common complications are hemoptysis, catheterization site bleeding and reperfusion lung injury; however, major complications and/or death following percutaneous intervention are rare [[17], [18], [19]]. The survival of patients with FM depends on the underlying etiology, the severity of its complications and possibly due to the therapeutic approach.…”

Section: Discussionmentioning

confidence: 99%

Successful management of fibrosing mediastinitis with severe vascular compromise: Report of two cases and literature review

Argueta

Villafuerte

Castaneda-Nerio

et al. 2020

Respiratory Medicine Case Reports

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Fibrosing mediastinitis is a rare disorder characterized by the invasive proliferation of fibrous tissue within the mediastinum. This fibrosis can result in compression of intrathoracic structures including the pulmonary vasculature leading to clinical symptoms and complications like pulmonary hypertension. Here, we present two cases of young patients with fibrosing mediastinitis complicated by pulmonary artery stenosis requiring medical and endovascular management with excellent results.

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“…It presents with symptoms of cough, shortness of breath, and even hemoptysis, and it carries a high mortality rate (up to 30%). [3][4][5] The literature has referred to the condition as fibrotic mediastinitis or sclerosing mediastinitis. 5 Two forms of the disease exist-namely, the focal and the diffuse.…”

Section: Discussionmentioning

confidence: 99%

Endovascular Treatment of Bilateral Pulmonary Artery Stenoses and Superior Vena Cava Syndrome in a Patient with Advanced Mediastinal Fibrosis

Valentin¹,

Kuban²,

Ramanathan³

et al. 2016

Texas Heart Institute Journal

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1 In severe cases, it can progress to obstruction of the superior vena cava (SVC), pulmonary vessels, esophagus, or airways. Whereas SVC obstruction is relatively common, pulmonary artery (PA) involvement is less frequently encountered.2 Very few reports of long-term outcomes exist in the medical literature of either SVC or PA stents in this patient population. We discuss a case in which MF resulted in PA and SVC stenoses that were treated endovascularly, with early symptom improvement and 5-year survival with primary patency. Case ReportA 54-year-old man with histoplasmosis presented at our institution with worsening right-sided chest tightness and difficulty sleeping on his right side because of shortness of breath. Three years before this episode, he had developed SVC syndrome from progressive MF that we had successfully treated endovascularly with an SVC stent. Therefore, differential diagnosis for the current episode of dyspnea included restenosis of the SVC stent, pulmonary embolism, pneumonia, and pulmonary hypertension. A computed tomographic angiogram (CTA) revealed high-grade right-PA stenosis caused by continued progression of MF. In addition, imaging revealed near-complete occlusion of the truncus anterior to the right PA. Results of CTA also revealed that the SVC stent remained widely patent.Subsequently, we performed pulmonary arteriography to evaluate right-PA stenosis and PA hypertension, and to judge the possibility of intervention. Using a femoral approach, we advanced a Mott pulmonary catheter over the wire and into the right ventricle. The catheter was next advanced into the main PA, where the pressure was 35/12 mmHg, and then into the left PA-where digital subtraction arteriography revealed no abnormalities. The left main PA pressure was 36/10 mmHg. The catheter was then modified to make it more amenable to entering the stenosed right main PA. We used a 260-cm, modified stiff Glidewire ® (Terumo Medical Corporation; Somerset, NJ), advancing the wire and catheter into the right main PA. Digital subtraction arteriography revealed approximately 75% stenosis at the origin of the right main PA, extending distally 3.6 cm (Fig. 1). A pressure recording obtained distal to the obstruction was 11/5 mmHg (pressure gradient, 22 mmHg). Given the patient's symptoms, imaging findings, and pressure gradient, intervention was judged necessary.

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A pressing matter—mediastinal fibrosis with near obliteration of the pulmonary arteries

Cited by 4 publications

References 9 publications

Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach

Severe fibrosing mediastinitis with atypical presentation: Effective control with novel therapeutic approach

Successful management of fibrosing mediastinitis with severe vascular compromise: Report of two cases and literature review

Endovascular Treatment of Bilateral Pulmonary Artery Stenoses and Superior Vena Cava Syndrome in a Patient with Advanced Mediastinal Fibrosis

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