Radiologic-pathologic correlationLife-threatening hemoptysis caused by chronic idiopathic pulmonary hilar fibrosis with unilateral pulmonary vein occlusion
Introduction
Mediastinal fibrosis (MF), also called fibrosing mediastinitis, is a rare chronic inflammatory condition resulting in an infiltrative tumor-like mass in the mediastinum that may compress vital structures with associated symptoms. The cause of MF, which usually runs a progressive cause, is generally unknown (idiopathic MF) although Histoplasma infection and, less frequently, tuberculosis have been shown to underlie a significant number of cases [1], [2], [3], [4]. Therapy for MF has been disappointing, with many cases having a fatal outcome. A limited variant of MF, called chronic idiopathic pulmonary hilar fibrosis (CIPHF), has been described [5]. In contrast to the more common form of MF, surgical intervention appears to be beneficial and the prognosis is more favorable in CIPHF.
We describe the case of a patient who presented with a life-threatening hemoptysis caused by complete occlusion of the right pulmonary vein. The features of the disease were consistent with the CIPHF variant of MF.
Section snippets
Case report
A 47-year-old woman was admitted for severe hemoptysis and atrial fibrillation with cardiac failure. Her previous medical history included 2 episodes of pleurisy of unknown cause 5 and 16 years before her current admission and hysterectomy 8 years before the said admission. Six years before the current admission, a regurgitant mitral valve had been replaced with a St. Jude prosthetic device. During the implantation, it was noted that the superior vena cava was markedly stenosed at its junction
Discussion
Compression of the large pulmonary vessels and bronchi is a well-recognized complication of MF [2], [6], [7], [8], [9], [10]. In a review by Berry et al [6], 11 of 140 patients had pulmonary vein stenosis, whereas tracheobronchial involvement was seen more commonly. However, the most common presentation of MF is stenosis of the superior vena cava with its associated syndrome [6]. Depending on the involved structures, other manifestations may include dyspnea, hemoptysis, and cough [6], [11].
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Clinical Relevance of Computed Tomography Pulmonary Venography
2016, Heart Lung and CirculationCitation Excerpt :Factors causing pulmonary venous stenosis are listed in Table 1. Typical symptoms and signs of pulmonary venous stenosis are shortness of breath, chronic cough, recurrent haemoptysis, pulmonary oedema, recurrent pleural effusions and pulmonary venous hypertension [3–7]. Common radiological findings are thickened interseptal lines, ground-glass opacity, enlarged hilar lymph nodes and pleural effusions.
Case no 2
2009, Journal de RadiologiePulmonary Vein Total Occlusion Following Catheter Ablation for Atrial Fibrillation. Clinical Implications After Long-Term Follow-Up
2006, Journal of the American College of CardiologyCitation Excerpt :Hence, the venous drainage of the affected segment becomes mainly dependent on the ipsilateral veins draining the healthy lobes. If the ipsilateral vein(s) is also stenosed, the impedance to the pulmonary flow increases, adding to the hemodynamic burden and the resulting lung pathology (16,17). Therefore, in case of progression to total occlusion, we found that evaluating the ipsilateral vein involved in this compensatory mechanism is of utmost importance for timely intervention.
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