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Figure 1 Pediatric Pulmonary Arterial Hypertension (PAH) Treatment Guidelines. *"Acute responder" was defined as: 1) 20% decrease in mean pulmonary artery pressure; 2) no change or an increase in cardiac index; and 3) no change or a decrease in the ratio of pulmonary vascular resistance to systemic vascular resistance in response to vasodilator testing. When bosentan became available (April 2001). CCB = calcium channel blockade; RHF = right heart failure.