Pulmonary Hypertension
Conditions
Keywords
pediatric, pulmonary hypertension, real-world, epidemiology, outcome
Brief summary
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.
Interventions
None listed
Sponsors
Guangdong Provincial People's Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
No minimum to 18 Years
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| death | From enrollment to the event, assessed through study completion (target follow-up duration of 10 years; extended follow-up available for earlier enrollees) |
| transplantation | From enrollment to the event, assessed through study completion (target follow-up duration of 10 years; extended follow-up available for earlier enrollees) |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| PH-related hospitalization | From enrollment to first occurrence of the event, assessed through study completion (continue to be followed after event occurrence until primary outcome or study completion) | — |
| atrial septostomy | From enrollment to first occurrence of the event, assessed through study completion (continue to be followed after event occurrence until primary outcome or study completion) | — |
| Potts shunt | From enrollment to first occurrence of the event, assessed through study completion (continue to be followed after event occurrence until primary outcome or study completion) | — |
| clinical worsening | From enrollment to first occurrence of the event, assessed through study completion (continue to be followed after event occurrence until primary outcome or study completion) | Clinical worsening is defined as the first occurrence of death, transplantation, hospitalization for PH, atrial septostomy or Potts shunt, with need for escalation of PH-targeted therapy, or worsening functional status. |
| decline in WHO functional class | From enrollment to first occurrence of the event, assessed through study completion (continue to be followed after event occurrence until primary outcome or study completion) | — |
Countries
China
Contacts
CONTACTJie-Xin Zhang, Dr.
STUDY_CHAIRZhi-Cheng Jing, Prof.
Guangdong Provincial People's Hospital
STUDY_DIRECTORShu-Shui Wang, Prof.
Guangdong Provincial People's Hospital
STUDY_DIRECTORJie-Xin Zhang, Dr.
Guangdong Provincial People's Hospital
Outcome results
None listed