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Inoperable Chronic Thromboembolic Pulmonary Hypertension : Treatable With Medical Therapy

Nicholas S. Hill, MD, FCCP; Ioana R. Preston, MD, FCCP; Kari E. Roberts, MD
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Boston, MA

Correspondence to: Nicholas S. Hill, MD, FCCP, Division of Pulmonary, Critical Care and Sleep Medicine, Tufts-New England Medical Center, 750 Washington St, No. 257, Boston, MA 02111; e-mail nhill@tufts-nemc.org



Chest. 2008;134(2):221-223. doi:10.1378/chest.08-0482
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Chronic thromboembolic pulmonary hypertension (CTEPH) has long been considered to be the one “curable” form of pulmonary arterial hypertension (PAH) if amenable to pulmonary thromboendarterectomy (PEA).1 Estimated to occur in up to 4% of patients within 2 years after an episode of acute thromboembolism,2 CTEPH is probably underdiagnosed. In a 2003 update on their experience with 1,500 cases of PEA, Jamieson et al3 reported excellent long-term outcomes and a surgical mortality rate of 4.4% in their last 500 cases. Until recently, however, the potential responsiveness of patients with CTEPH to medical therapy had not been appreciated. During the mid-1990s, none other than the late authority on the subject, Ken Moser, responded rhetorically to a query from one of the authors of this editorial (N.H.) as follows: “Why would we use vasodilator therapy for CTEPH?—it’s a mechanical problem.” To be fair, only IV epoprostenol was then available for therapy, and the responses to the traditional vasodilators, including calcium channel blockers, had been disappointing.

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