Imatinib for the Treatment of Pulmonary Arterial Hypertension

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Abstract
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 1056 dyn · sec · cm–5 (with a mean pulmonary-artery pressure of 59 mm Hg and a cardiac index of 2.08 liters per minute per square meter) to 1538 dyn · sec · cm–5 (with a mean pulmonary-artery pressure of 58 mm Hg and a cardiac index of 1.36 liters per minute per square meter). At this point, the patient was in New York Heart Association (NYHA) functional class IV ( Figure 1 ). During preparations for lung transplantation, the patient refused intravenous prostanoid treatment for bridging to this procedure. Other causes of deterioration (including occult neoplasms) were ruled out by diagnostic measures (e.g., whole-body computed tomography, bone marrow aspiration, and assessment of serum tumor markers).