IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Pulmonary Artery Aneurysms Ali Ataya and Hassan Alnuaimat Department of Pulmonary and Critical Care Medicine, University of Florida, Gainesville, Florida
Figure 1. Axial maximum intensity projection chest computed tomography scan showing pulmonary artery aneurysms (arrows) and evidence of bilateral pleural effusions, larger on the left.
Figure 3. A three-dimensional reconstructed image of the heart and pulmonary vasculature showing bilateral pulmonary artery aneurysms (arrows).
Figure 2. Coronal maximum intensity projection chest computed tomography scan showing pulmonary artery aneurysms (arrows) and evidence of bilateral pleural effusions, larger on the left.
Figure 4. Axial maximum intensity projection chest computed tomography scan 2 years after treatment showing complete resolution of the pulmonary artery aneurysms and pleural effusions.
Am J Respir Crit Care Med Vol 190, Iss 7, pp e26–e27, Oct 1, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201401-0113IM Internet address: www.atsjournals.org
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American Journal of Respiratory and Critical Care Medicine Volume 190 Number 7 | October 1 2014
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 5. Coronal maximum intensity projection chest computed tomography scan 2 years after treatment showing complete resolution of the pulmonary artery aneurysms and pleural effusions.
A 32-year-old Hispanic man with a recent diagnosis of dural venous thrombosis presents with acute hypoxic respiratory failure and fevers while on warfarin. A computed tomography chest angiogram showed evidence of bilateral pulmonary artery aneurysms with associated bilateral pulmonary embolisms (Figures 1 and 2), superior and inferior vena cava thrombosis, and bilateral pleural effusions. A three-dimensional reconstruction of the pulmonary vasculature was done that showed the bilateral pulmonary aneurysms (Figure 3). There was also evidence of intrathoracic and extrathoracic collateral vessels secondary to the superior vena cava thrombosis. A transthoracic echocardiogram revealed a right atrial thrombus, and Doppler ultrasound studies showed bilateral internal jugular and subclavian vein and femoral vein thrombosis. Thoracentesis performed revealed serosanguineous fluid that was consistent with chylothoraces on analysis. Workup for a hypercoagulable state and heparin-induced thrombocytopenia were negative. Based on the clinical and radiological presentation, he was diagnosed with Hughes-Stovin syndrome and was treated with pulse dose steroids followed by cyclophosphamide for 6 months, then transitioned to azathioprine for 1.5 years. He was continued on warfarin during this time. After 2 years, follow-up imaging revealed complete resolution of bilateral pulmonary artery aneurysms and no evidence of thromboembolic disease recurrence (Figures 4 and 5). Hughes-Stovin syndrome is a rare and usually fatal disorder that is considered to be a cardiovascular variant of Behçet disease. Early diagnosis and treatment is important, because patients are at a high risk for devastating pulmonary artery aneurysm rupture (1). Treatment usually involves immunosuppressive agents and anticoagulation therapy (2). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Khalid U, Saleem T. Hughes-Stovin syndrome. Orphanet J Rare Dis 2011;6:15.
2. Lee J, Noh JW, Hwang JW, Kim H, Ahn JK, Koh EM, Cha HS. Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient. Clin Rheumatol 2008;27:1455–1458.
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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Figure 1. Axial maximum intensity projection chest computed tomography scan showing pulmonary artery aneurysms (arrows) and evidence of bilateral pleural effusions, larger on the left.
Figure 3. A three-dimensional reconstructed image of the heart and pulmonary vasculature showing bilateral pulmonary artery aneurysms (arrows).
Figure 2. Coronal maximum intensity projection chest computed tomography scan showing pulmonary artery aneurysms (arrows) and evidence of bilateral pleural effusions, larger on the left.
Figure 4. Axial maximum intensity projection chest computed tomography scan 2 years after treatment showing complete resolution of the pulmonary artery aneurysms and pleural effusions.
Am J Respir Crit Care Med Vol 190, Iss 7, pp e26–e27, Oct 1, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201401-0113IM Internet address: www.atsjournals.org
e26
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 7 | October 1 2014
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES
Figure 5. Coronal maximum intensity projection chest computed tomography scan 2 years after treatment showing complete resolution of the pulmonary artery aneurysms and pleural effusions.
A 32-year-old Hispanic man with a recent diagnosis of dural venous thrombosis presents with acute hypoxic respiratory failure and fevers while on warfarin. A computed tomography chest angiogram showed evidence of bilateral pulmonary artery aneurysms with associated bilateral pulmonary embolisms (Figures 1 and 2), superior and inferior vena cava thrombosis, and bilateral pleural effusions. A three-dimensional reconstruction of the pulmonary vasculature was done that showed the bilateral pulmonary aneurysms (Figure 3). There was also evidence of intrathoracic and extrathoracic collateral vessels secondary to the superior vena cava thrombosis. A transthoracic echocardiogram revealed a right atrial thrombus, and Doppler ultrasound studies showed bilateral internal jugular and subclavian vein and femoral vein thrombosis. Thoracentesis performed revealed serosanguineous fluid that was consistent with chylothoraces on analysis. Workup for a hypercoagulable state and heparin-induced thrombocytopenia were negative. Based on the clinical and radiological presentation, he was diagnosed with Hughes-Stovin syndrome and was treated with pulse dose steroids followed by cyclophosphamide for 6 months, then transitioned to azathioprine for 1.5 years. He was continued on warfarin during this time. After 2 years, follow-up imaging revealed complete resolution of bilateral pulmonary artery aneurysms and no evidence of thromboembolic disease recurrence (Figures 4 and 5). Hughes-Stovin syndrome is a rare and usually fatal disorder that is considered to be a cardiovascular variant of Behçet disease. Early diagnosis and treatment is important, because patients are at a high risk for devastating pulmonary artery aneurysm rupture (1). Treatment usually involves immunosuppressive agents and anticoagulation therapy (2). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Khalid U, Saleem T. Hughes-Stovin syndrome. Orphanet J Rare Dis 2011;6:15.
2. Lee J, Noh JW, Hwang JW, Kim H, Ahn JK, Koh EM, Cha HS. Successful cyclophosphamide therapy with complete resolution of pulmonary artery aneurysm in Hughes-Stovin syndrome patient. Clin Rheumatol 2008;27:1455–1458.
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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