Unilateral pulmonary vein stenosis secondary to idiopathic fibrosing mediastinitis

Leong, Derek; Dundon, Benjamin K.; Steele, Peter

doi:10.1136/hrt.2007.124404

Cited by 9 publications

(6 citation statements)

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“…It is thought to occur due to exposure to the Histoplasma capsulatum antigen within the mediastinum, triggering an intense inflammatory host response resulting in proliferation and invasion of fibrous tissue into vital structures [3] , [4] . One complication of the fibrotic reaction is pulmonary vein (PV) stenosis, previously described in case reports and series [1] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] . Diagnosis of PV stenosis is challenging due to its gradual onset of nonspecific symptoms including fatigue and dyspnea, and thus presentation is typically delayed.…”

Section: Introductionmentioning

confidence: 99%

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Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience

Ponamgi

DeSimone

Lenz

et al. 2015

IJC Heart & Vasculature

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IntroductionFibrosing mediastinitis (FM) is a rare but fatal disease characterized by an excessive fibrotic reaction in the mediastinum, which can lead to life-threatening stenosis of the pulmonary veins (PV). Catheter-based intervention is currently the only viable option for therapy. However, the current literature on how best to manage these difficult cases, especially in regards to sequential interventions and their potential complications is very limited.MethodsWe searched through a database of all patients who have undergone PV interventions at the Earl H. Wood Cardiac Catheterization Laboratory in Mayo Clinic, Rochester. From this collection, we selected patients that underwent PV intervention to relieve stenosis secondary to FM.ResultsEight patients were identified, with a mean age of 41 years (24–59 years). Five were men, and three were women. Three patients underwent balloon angioplasty alone, and five patients had stents placed. The majority of patients had acute hemodynamic and symptomatic improvement. More than one intervention was required in five patients, four patients had at least one episode of restenosis, and four patients died within four weeks of their first PV intervention.ConclusionsWe describe the largest reported case series of catheter-based intervention for PV stenosis in FM. Although catheter-based therapy improved hemodynamics, short-term vascular patency, and patient symptoms, the rate of life-threatening complications, restenosis, and mortality associated with these interventions was found to be high. Despite these associated risks, catheter-based intervention is the only palliative option available to improve quality of life in severely symptomatic patients with PV stenosis and FM. Patients with PV stenosis and FM (especially those with bilateral disease) have an overall poor prognosis in spite of undergoing these interventions due to the progressive and recalcitrant nature of the disease. This underscores the need for further innovative approaches to manage this disease.

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Section: Introductionmentioning

confidence: 99%

“…Relieving obstruction with timely angioplasty in PV stenosis caused by other etiologies (i.e. pulmonary vein isolation for atrial fibrillation) normalizes venous flow into the left atrium [19] , [20] ; however, there is limited data on how best to manage PV stenosis caused by FM [1] , [5] , [6] , [7] , [21] .…”

Section: Introductionmentioning

confidence: 99%

Catheter-based intervention for pulmonary vein stenosis due to fibrosing mediastinitis: The Mayo Clinic experience

Ponamgi

DeSimone

Lenz

et al. 2015

IJC Heart & Vasculature

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“…Der Befall von Herz und Gefäßen kann zur Obstruktion von Pulmonalarterien oder Pulmonalvenen, VCS-Syndrom oder zu einer konstriktiven Perikarditis führen [2,12,25]. Ein VCS-Syndrom entwickelt sich typischerweise über mehrere Jahre, weshalb aufgrund des dann ausgedehnten Kollateralkreislaufs die klinischen Symptome eher diskret sind [6].…”

Section: Klinische Präsentationunclassified

Mediastinalfibrose

Sackmann¹

2009

Pneumologe

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“…In sarcoidosis, two cases were reported by HAMILTON-CRAIG et al [7] who were successfully treated by stent placement in the pulmonary arteries. Conversely, there are only a few cases in the literature of pulmonary venous obstruction [10][11][12][13]. This happened in three of 64 patients (4.6%) with fibrosing mediastinitis in the study by SCHOWENGERDT et al [11].…”

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confidence: 98%

“…Regarding sarcoidosis, DEVARAJ et al [2] reported two cases in a series of 12 patients, with CT images and histology of fibrosing mediastinitis (16.6% of patients). Vascular obstruction due to fibrosing mediastinitis is frequent [3][4][5][6][7][8][9][10][11][12][13], particularly during histoplasmosis. Superior vena cava obstruction is the most commonly reported and is easily diagnosed.…”

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confidence: 99%