Abstract

Pulmonary arterial hypertension (PAH) is a life-threatening disease, the prognosis of which has changed dramatically in the past decade since the introduction of new therapeutic agents and the off-label application of adult pulmonary hypertension (PH)-specific therapies to children.1–3 However, PAH still has no cure, and the aim of treatment is to prolong survival by improving quality of life, symptoms, exercise capacity, and hemodynamics. The selection of appropriate therapies for pulmonary hypertension is complex, and they must be carefully chosen according to the etiology and pulmonary vasoreactivity.3 As insight advances into mechanisms responsible for the development of PAH, we hope the introduction of novel therapeutic agents will further improve the outcome of this disease.

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