This Article Full Text (PDF) Data Supplement Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kindermann, M. Articles by Böhm, M. Search for Related Content PubMed PubMed Citation Articles by Kindermann, M. Articles by Böhm, M. Pubmed/NCBI databases Substance via MeSH Medline Plus Health Information Aneurysms Thrombophlebitis Vasculitis Related Collections Deep vein thrombosis Thrombosis risk factors

(Circulation. 2003;108:e156.)
© 2003 American Heart Association, Inc.

Images in Cardiovascular Medicine

Hughes-Stovin Syndrome

Michael Kindermann, MD; Heinrike Wilkens, MD; Wilfried Hartmann, MD; Hans-Joachim Schäfers, MD; Michael Böhm, MD

From the Department of Internal Medicine, Division of Cardiology and Angiology, University of the Saarland, Homburg, Germany (M.K., M.B.); Department of Internal Medicine, Division of Pulmology, University of the Saarland, Homburg, Germany (H.W.); Department of Internal Medicine, Clinic for Respiratory Diseases, Hospital Bremen Ost, Germany (W.H.); and Department of Thoracic and Cardiovascular Surgery, University of the Saarland, Homburg, Germany (H.-J.S.).

Correspondence to Michael Kindermann, MD, Innere Medizin III (Kardiologie/Angiologie), Universitätskliniken des Saarlandes, Kirrberger Straße, D 66421 Homburg/Saar, Germany. E-mail Michael.Kindermann{at}T-Online.de

A50-year-old woman suffering from shortness of breath and unproductive coughing for 6 months was admitted to the hospital because of recurrent hemoptysis. The patient had a history of deep venous thrombosis of both legs and the pelvic veins. A thrombophilic state was assumed because of a factor V Leiden mutation.

Angiography showed occlusion of both iliac vein systems and of the vena cava inferior (Figure 1). Venous access to the central circulation was possible through the superior caval vein. Although pulmonary angiography of the left lung showed some enlargement at the origin of the left upper-lobe pulmonary artery, right-sided pulmonary angiography revealed two large aneurysms originating from the right upper-lobe artery and the intermediate branch of the pulmonary artery (Figure 2). The intermediate artery aneurysm was resected, and the artery was reconstructed using a saphenous vein graft. The patient also underwent right upper lobectomy because the right upper-lobe aneurysm reached too far into the periphery to allow reconstruction. Histological examination of the specimen showed vasculitis, and immunosuppressive therapy was started.

View larger version (185K): [in this window] [in a new window]   Figure 1. Injection of dye through a right femoral access shows occlusion of both the iliac veins and the inferior caval vein. Blood is drained by a collateral circulation of pelvic and lumbar veins (see Movie I).

View larger version (178K): [in this window] [in a new window]   Figure 2. Right-sided pulmonary angiography. Two large aneurysms originating from the upper lobe and intermediate pulmonary arteries. Marked hypoperfusion of the right upper and lower lobe is evident (see Movie II).

The Hughes-Stovin syndrome consists of deep venous thrombosis often involving the caval vein accompanied by single or multiple pulmonary arterial aneurysms. Massive pulmonary hemorrhage caused by rupture of an aneurysm is a frequent terminal event. Patients affected by this syndrome are often young adult men in the second to fourth decade of life. Case reports describing women with Hughes-Stovin syndrome are rare.

Footnotes

Movies I and II are available in the online-only Data Supplement at http://www.circulationaha.org.

The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.

This article has been cited by other articles:

				J. T. Willerson December 9, 2003 Circulation, December 9, 2003; 108(23): 2827 - 2827. [Full Text] [PDF]

This Article Full Text (PDF) Data Supplement Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kindermann, M. Articles by Böhm, M. Search for Related Content PubMed PubMed Citation Articles by Kindermann, M. Articles by Böhm, M. Pubmed/NCBI databases Substance via MeSH Medline Plus Health Information Aneurysms Thrombophlebitis Vasculitis Related Collections Deep vein thrombosis Thrombosis risk factors