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J Thorac Cardiovasc Surg 2005;130:213-215
© 2005 The American Association for Thoracic Surgery

Brief Communication

Aortic root replacement in a patient with pulmonary dysfunction caused by severe chest deformity associated with Marfan syndrome

Iki Adachi, MD a , Hitoshi Ogino, MD a , * , Hideaki Imanaka, MD b , Hitoshi Matsuda, MD a , Kenji Minatoya, MD a , Hiroaki Sasaki, MD a

a Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
b Intensive Care Unit, National Cardiovascular Center, Osaka, Japan

Received for publication January 3, 2005; revisions received January 15, 2005; accepted for publication February 8, 2005.

* Address for reprints: Hitoshi Ogino, MD, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan (Email: hogino@hsp.ncvc.go.jp).

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

Great advances in respiratory management have recently widened the indication for cardiovascular surgery for patients with pulmonary dysfunction. We describe a patient with an extremely low vital capacity caused by severe chest deformity who tolerated aortic root replacement as a result of adequate respiratory management, including bilevel positive airway pressure (BiPAP) ventilation.

Clinical Summary

A 39-year-old man with Marfan syndrome was scheduled to undergo aortic root replacement for annuloaortic ectasia and aortic insufficiency. He had been receiving home oxygen therapy for 10 years and nocturnal use of nasal continuous positive airway pressure ventilation for 1 year because of frequent episodes of respiratory failure.

On admission, he showed respiratory distress at grade IV of the Hugh-Jones classification. Spirometry showed a severe restrictive pattern: vital capacity of 1080 mL (28% of predicted value) and forced expiratory volume in 1 second of 890 mL. Arterial blood gases (ABGs) under 1 L of oxygen inhalation revealed metabolically compensated hypercapnia: pH, 7.41; PaCO 2. . . [Full Text of this Article]




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