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Background-Pulmonary arterial hypertension (PAH) is a progressive and fatal disease. Sildenafil is a type 5 phosphodiesterase inhibitor and pulmonary vasodilator. Therefore, we hypothesized that sildenafil would improve distance walked in 6 minutes and hemodynamics in children with PAH. Methods and Results-After baseline assessment of hemodynamics by cardiac catheterization and distance walked in 6 minutes, we administered oral sildenafil at 0.25 to 1 mg/kg 4 times daily to 14 children (median age, 9.8 years; range, 5.3 to 18). Diagnoses were primary (nϭ4) and secondary (nϭ10) PAH. We repeated the 6-minute walk test at 6 weeks and at 3, 6, and 12 months (nϭ14) and cardiac catheterization (nϭ9) after a median follow-up of 10.8 months (range, 6 to 15.3). During sildenafil therapy, the mean distance walked in 6 minutes increased from 278Ϯ114 to 443Ϯ107 m over 6 months (Pϭ0.02), and at 12 months, the distance walked was 432Ϯ156 m (Pϭ0.005

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