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Clinical Decisionmaking

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Paget-Schroetter syndrome should be considered whenever a person with repetitive arm motion has pulmonary hypertension

When patients with repetitive arm motion, such as tennis players or hairdressers, have pulmonary hypertension, thrombosis of the axillary veins under the clavicle—also called Paget-Schroetter syndrome—should be suspected. This syndrome has been associated with exertion of the arms and is also called "effort thrombosis." Sanjay Saint, M.D., M.P.H., of the University of Michigan, and colleagues describe the case of a 17-year-old athlete—a discus thrower on his school's track team—whose athletic efforts could certainly have contributed to compression of the subclavian vein and thrombosis (blood clots) in his arms that traveled to his lungs (pulmonary embolism).

The researchers point out that these diagnoses are not easy, especially in seemingly robust adolescents. The diagnosis in this case was most likely delayed because a physically robust patient had atypical signs and an uncommon disease. Pulmonary embolism is rare in children and adolescents (7.8 per 10,000 hospital admissions for adolescents or young adults). Most patients have symptoms of venous obstruction such as pain, swelling, and bluish discoloration. Nonocclusive thromboses, as seen in this patient, may not have local symptoms, instead becoming symptomatic only after pulmonary embolization.

For example, this patient had no jugular venous distention even at the time when his right ventricular and pulmonary arterial pressures were markedly elevated. Catheter-directed thrombolytic therapy followed by decompression of the thoracic outlet has become the standard of care for Paget-Schroetter syndrome. Outcomes are better with early thrombolytic therapy, according to Dr. Saint, whose research is supported in part by the Agency for Healthcare Research and Quality (HS11540).

See "The unusual suspect," by Sandra Bliss, M.D., Steven Weinberger, M.D., Mark Meider, M.D., and Dr. Saint, in the December 5, 2002, New England Journal of Medicine 347(23), pp. 1876-1881.

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