H.H Leuchte, MD; R.A Baumgartner; J Behr, MD
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Leuchte H, Baumgartner R, Behr J. Treatment of Severe Pulmonary Hypertension with Inhaled Iloprost. Ann Intern Med. 2003;139:306. doi: 10.7326/0003-4819-139-4-200308190-00025
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Published: Ann Intern Med. 2003;139(4):306.
TO THE EDITOR:
We report successful treatment of severe pulmonary hypertension with inhaled aerosolized iloprost in a patient with Gaucher disease, a lysosomal storage disorder (1-4). A 39-year-old woman with underlying adult Gaucher disease was admitted to our hospital with exertional dyspnea that markedly limited her physical activity (New York Heart Association class III). She had received a diagnosis of type I Gaucher disease at the age of 12 years and had been treated with enzyme replacement therapy (imiglucerase).
On presentation, she was able to walk 500 m in 6 minutes. During cycle spiroergometry with a working rate of 60 W, she reached a peak oxygen uptake (VO2) of 15.1 mL/kg per minute. Brain natriuretic peptid levels were increased 10-fold. Initial right-heart catheterization showed severe precapillary pulmonary hypertension with pulmonary vascular resistance of 1506 dynes/s · cm −5, mean pulmonary artery pressure of 61 mm Hg, and cardiac output of 2.71 L/min. Acute testing with inhaled iloprost showed substantial reduction in pulmonary vascular resistance (32%). The patient received oral anticoagulant therapy and was treated with inhaled iloprost, 100 µg/d, for 9 months.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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