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Abstract Idiopathic pulmonary arterial hypertension (PAH) in infants is a rare, life-threatening condition characterized by elevated pulmonary artery pressure resulting from endothelial dysfunction, vasoconstriction, and vascular remodeling. The treatment options are limited, and the prognosis is poor. In children, 5-year survival following diagnosis remains suboptimal, with estimates of approximately 74% despite advances in therapy.
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