Pulmonary hypertension in pediatrics: from clinical suspicion to management

Pediatric pulmonary hypertension differs from adult pulmonary hypertension in many ways, including multifactorial etiologies and comorbidities that can impact diagnosis, response to therapy, and outcome. The main etiologies of pediatric PH are idiopathic pulmonary arterial hypertension (PAH), PAH associated with congenital heart disease (PAH-CDH) and developmental lung disorders. Thorough diagnostic evaluation is necessary to properly classify pulmonary hypertension, find a potential treatable cause, and guide therapy. Diagnosis still relies on invasive hemodynamics that require sedation in most children. Management of pediatric pulmonary hypertension is mainly guided by small-scale studies, expert opinion, and extrapolation of adult data considering the paucity of trials in this population. The aim of this review is to provide an up-to-date summary of current knowledge on pediatric pulmonary hypertension, covering diagnosis to management, and to highlight the key takeaways from the pediatric task force of the 7th World Symposium on Pulmonary Hypertension, particularly regarding classification modifications, risk stratification, and management. What is known: • Pediatric pulmonary hypertension is a rare condition, with the main etiologies being idiopathic, associated with congenital heart disease and developmental lung disorders. • A risk-oriented treatment approach is recommended, with lower-risk mortality as the therapeutic target. Treatment should be escalated if the treatment response is unsatisfactory. What is new: • Classification of pulmonary arterial hypertension associated with congenital heart disease is expanded beyond the concept of a shunt. • Risk stratification is refined through the use of 25 validated risk factors.