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Naotaka Motoyoshi
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J Thorac Cardiovasc Surg 2009;137:1282-1284
© 2009 The American Association for Thoracic Surgery

Brief Communication

Pulmonary venous obstruction in a patient with Marfan syndrome: Rare presentation of an expanding dissecting descending thoracic aortic aneurysm with annuloaortic ectasia

Yoshikatsu Saiki, MD, PhD*, Shunsuke Kawamoto, MD, PhD, Junetsu Akasaka, MD, PhD, Naotaka Motoyoshi, MD, PhD, Sadahiro Sai, MD, PhD, Koichi Tabayashi, MD, PhD

Department of Cardiovascular Surgery, Graduate School of Medicine, Tohoku University, Sendai, Japan

Received for publication December 23, 2007; accepted for publication January 12, 2008.

* Address for reprints: Yoshikatsu Saiki, MD, Department of Cardiovascular Surgery, Graduate School of Medicine, Tohoku University, Seiryomachi, Aoba-ku, Sendai, Japan 980-8574. (Email: ysaiki@mail.tains.tohoku.ac.jp).

The first 20% of the full text of this article appears below.

Pulmonary venous obstruction (PVO) is an extremely rare condition in patients with acquired cardiovascular disease. We describe the case of a patient with chronic dissecting descending thoracic aortic aneurysm and annuloaortic ectasia associated with Marfan syndrome in whom PVO developed. The patient underwent successful emergency single-stage repair of the extensive thoracic aortic aneurysm.

Clinical Summary

A 15-year-old female patient with Marfan syndrome had been followed up at a local hospital for annuloaortic ectasia with mild aortic insufficiency. A type B acute aortic dissection developed and was managed conservatively. Four months later, the patient had sudden onset of severe back pain, and computed tomographic (CT) scans revealed expansion of the dissecting descending thoracic aorta. She was admitted to our intensive care unit because rupture of the thoracic aorta appeared impending. Sixteen hours after admission, large amounts of foamy sputum were observed. Repeated chest radiographs revealed pulmonary venous congestion, specifically on the right side (Go Figure 1, A). A transthoracic echocardiogram revealed a moderate degree of aortic regurgitation and turbulent right pulmonary venous flow. On the basis of the CT scans, the PVO on the right side was . . . [Full Text of this Article]






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