Pediatric pulmonary hypertension (PH) shares similarities with PH in adulthood, but specific differences still require unique approaches. Despite major advances, risk scores for pediatric PH still need to be validated, and targeted drug therapies in pediatric populations remain under-studied. Consequently, there is a great need for comprehensive real-world longitudinal data of pediatric PH.

The INSPIRE-PH registry is a multicenter, observational, real-world registry designed to systematically collect longitudinal clinical data on children and adolescents with PH. Patients enrolled in the registry receive standard clinical care determined by their treating physicians. No study-mandated interventions are imposed. Longitudinal observation of this cohort aims to provide important insights into disease progression, treatment effectiveness, and long-term prognosis in pediatric PH.

The cohort was initially established prospectively in 2006, with all cases reclassified according to the 7th World Symposium for Pulmonary Hypertension (WSPH) criteria in 2024, and new patients continue to be prospectively enrolled. The registry is supervised by the principal investigator, independently from the financial sponsors.

Inclusion Criteria:

• Children and adolescents aged <18 years at diagnosis
• Diagnosed pulmonary hypertension (PH) according to the prevailing diagnostic criteria at the time of enrollment
• Enrollment in the registry at participating centers
• Provision of informed consent by patients and/or legal guardians
• Availability for longitudinal follow-up

Exclusion Criteria:

• Inability to confirm PH diagnosis due to insufficient clinical or hemodynamic data
• Refusal or inability to provide informed consent

This registry includes children and adolescents diagnosed with PH who are enrolled at participating centers. Patients are followed longitudinally with standardized collection of clinical, functional, imaging and hemodynamic data, with treatment strategies in a real-world setting, allowing evaluation of epidemiology, disease progression, and long-term outcomes.