Imatinib for the Treatment of Pulmonary Arterial Hypertension

Abstract

To the Editor: We report on a 61-year-old man with a rapidly progressing form of familial idiopathic pulmonary arterial hypertension. Five years earlier, the patient had presented with signs of right-sided heart failure in our pulmonary-hypertension referral center. During the previous year, his condition had progressively deteriorated, despite advanced combination therapy with oral bosentan (125 mg twice daily),1 inhaled iloprost (nine inhalations per day),2 and oral sildenafil (50 mg three times a day).3,4 The patient's six-minute walking distance had progressively declined during the previous nine months, from 323 m to 260 m, and the pulmonary vascular resistance increased from . . .