ABSTRACT
Similar to adults, pulmonary arterial hypertension is defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest, with a normal pulmonary artery wedge pressure less than 15 mm Hg and an increased pulmonary
Introduction 615 Definition 615 Epidemiology 616 Heritability 616 Evaluation 617 Conventional therapy 618 Significant comorbidities 619 Vasoreactivity testing 619 Pharmacologic therapy of pulmonary arterial
hypertension 619
Calcium channel blockers 619 Prostacyclins 621 Endothelin receptor antagonists 621 Phosphodiesterase-5 inhibitors and soluble guanylate
cyclase stimulators 622 Combination therapy 623 Atrial septostomy and Potts shunt for refractory
pulmonary arterial hypertension 623 Transplantation 624 References 624
vascular resistance greater than 3 Wood units/M2 (6,18). The Nice classification of PAH is appropriate for both adults and children (6,19). In younger children, the pulmonary arterial pressure is frequently referenced as a ratio to systemic arterial pressure, with a significant difference considered greater than 0.5. In both adults and children, the diagnosis of IPAH requires the exclusion of all possible etiologies of associated pulmonary arterial hypertension, left heart disease, and respiratory disease.
