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Imatinib Mesylate in the Treatment of Refractory Idiopathic Pulmonary Arterial Hypertension
- Karen C. Patterson, MD;
- Ariel Weissmann, MD, MPH;
- Tahamtan Ahmadi, MD, PhD; and
- Harrison W. Farber, MD
- From Boston Medical Center, Boston, MA 02118.
Background: Idiopathic pulmonary arterial hypertension (PAH) is a pathologic process of inappropriate vasoconstriction, vascular remodeling with smooth muscle cell hypertrophy, and in situ thrombosis, which eventually leads to right-heart failure and death. Current treatments primarily attenuate vasoconstriction and do not substantially reduce morbidity and mortality rates.
Objective: To describe a patient with severe, refractory idiopathic PAH that did not respond to conventional treatment but did show clinical and hemodynamic response to imatinib mesylate therapy, which may affect vascular remodeling.
Case Report: A 52-year-old man with rapidly progressive idiopathic PAH was evaluated for progressive dyspnea on exertion. Echocardiography and right-heart catheterization confirmed PAH; mean pulmonary artery pressure was 66 mm Hg. No cause for PAH was found. On the basis of his symptoms, the patient was categorized into World Health Organization functional class III. Therapy with furosemide and intravenous treprostinil (1) was started. …
