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Successful Pulmonary Thromboendarterectomy in a Patient With Klippel-Trenaunay Syndrome*

Beat Walder, MD; David P. Kapelanski, MD; William R. Auger, MD, FCCP; Peter F. Fedullo, MD, FCCP
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*From the Divisions of Pulmonary and Critical Care Medicine (Drs. Walder, Auger, and Fedullo) and Cardiothoracic Surgery (Dr. Kapelanski), University of California, San Diego, School of Medicine, and UCSD Medical Center, San Diego, CA.

Correspondence to: Peter F. Fedullo, MD, FCCP, Division of Pulmonary and Critical Care Medicine, University of California, San Diego, School of Medicine, UCSD Medical Center, San Diego, CA; e-mail: bwalder@ucsd.edu



Chest. 2000;117(5):1520-1522. doi:10.1378/chest.117.5.1520
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Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by a triad of cutaneous vascular nevi, soft tissue or bony hypertrophy, and varicose veins or venous malformations involving one or more extremities. An incidence of venous thromboembolism of up to 22% has been reported in this disorder. Also reported is the development of chronic thromboembolic pulmonary hypertension (CTEPH) and subsequent death from right ventricular failure. We report the first patient with KTS to undergo a successful pulmonary thromboendarterectomy for CTEPH.

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