Endovascular Stenting as a Treatment of Severe Pulmonary Hypertension Caused by Tumor Compression of the Main Pulmonary Arteries
Ingo Paetsch1, MD, Ralf Ewert1, MD, Felix Berger2, MD, Roland Hetzer3, MD, Eike Nagel1, MD.
Department of Internal Medicine, German Heart Institute Berlin1, Department of Congenital Heart Diseases, University of Zurich2 and Department of Cardiothoracic Surgery, German Heart Institute Berlin3.
A 31-year-old woman presented with worsening exertional dyspnea. Echocardiography showed dilatation of the right atrium and ventricle and an elevated pulmonary artery pressure (»115 mmHg, no valve disease) suggesting severe pulmonary hypertension (PHT). Cardiopulmonary exercise testing revealed a reduced maximal oxygen uptake (V02max 16.6 ml/min/kg (68% pred)) and impaired ventilatory efficiency (VE/VCO2 slope 44 (157% pred)). A computed tomography (CT) was performed, unexpectedly showing a mediastinal tumor (CT-guided biopsy: paraganglioma) with compression of the right pulmonary artery branch. CT as well as transesophageal echocardiography failed to visualize the whole course of the left main pulmonary artery. Thus, we performed magnetic resonance imaging (figure 1 a) combined with three dimensional contrast-enhanced MR angiography revealing additional high-grade stenosis of the left main pulmonary artery. These findings were confirmed by pulmonary X-ray angiography (figure 1 b). Pressure recordings: truncus pulmonalis 80/0(32) mmHg, right and left side post-stenotic pressure 9/4 and 13/5 mmHg, respectively.
Two endovascular stents were implanted into both main pulmonary arteries (figure 1b). Immediately after the implantation pulmonary artery pressure dropped dramatically (right main branch: 38/4 mmHg; left main branch 33/4 mmHg). Dyspnea ceased completely within the following days. At 6 week follow-up exercise testing demonstrated near-normal findings (V02max 22.3 ml/min/kg (91% pred), VE/VCO2 slope 30 (107% pred)).
In patients with pulmonary hypertension a careful diagnostic work-up including CT and MR imaging is crucial. To our knowledge, this is the first case of severe PHT due to tumor compression treated successfully with endovascular pulmonary stenting as a "bridge to operation".
Figure legend
Figure 1. a: Native (left) and postcontrast (right) MR anatomic imaging of the thorax (T1 weighting, transverse slice orientation) confirming CT finding of right pulmonary branch stenosis. Solid arrow: tumor mass of paraganglioma. Dotted arrow: obstruction of right pulmonary artery branch. Open arrow: malignant pleural effusion and lobar atelectasis. b: Conventional X-ray angiography Left: Bilateral pulmonary branch stenoses (arrows). Right: Angiographic result after implantation of endovascular stents in both pulmonary branches (arrows).
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