Insights Into Pediatric Pulmonary Hypertension: A Real-World Registry of Epidemiology and Outcomes (INSPIRE-PPH)

March 24, 2026 updated by: Zhi-Cheng Jing, MD, Guangdong Provincial People's Hospital

Insights Into Epidemiology, Management, and Outcomes of Pediatric Pulmonary Hypertension: The INSPIRE-PH Multicenter Real-World Registry

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.

Study Overview

Status

Recruiting

Conditions

Study Type

Observational

Enrollment (Estimated)

450

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Locations

  • China
    • Guangdong
      • Guangzhou, Guangdong, China, 510080
        • Recruiting
        • Guangdong Provincial People's Hospital

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Child
  • Adult

Accepts Healthy Volunteers

N/A

Sampling Method

Non-Probability Sample

Study Population

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.

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Time Frame
death
Time Frame: From enrollment to the event, assessed through study completion (target follow-up duration of 10 years; extended follow-up available for earlier enrollees)
From enrollment to the event, assessed through study completion (target follow-up duration of 10 years; extended follow-up available for earlier enrollees)
transplantation
Time Frame: From enrollment to the event, assessed through study completion (target follow-up duration of 10 years; extended follow-up available for earlier enrollees)
From enrollment to the event, assessed through study completion (target follow-up duration of 10 years; extended follow-up available for earlier enrollees)

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
PH-related hospitalization
Time Frame: 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)
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
Time Frame: 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)
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
Time Frame: 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)
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
Time Frame: 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.
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)
decline in WHO functional class
Time Frame: 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)
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)

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Guangdong Provincial People's Hospital

Investigators

  • Study Chair: Zhi-Cheng Jing, Prof., Guangdong Provincial People's Hospital
  • Study Director: Shu-Shui Wang, Prof., Guangdong Provincial People's Hospital
  • Study Director: Jie-Xin Zhang, Dr., Guangdong Provincial People's Hospital

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

January 4, 2006

Primary Completion (Estimated)

December 31, 2032

Study Completion (Estimated)

December 31, 2035

Study Registration Dates

First Submitted

March 18, 2026

First Submitted That Met QC Criteria

March 24, 2026

First Posted (Actual)

March 27, 2026

Study Record Updates

Last Update Posted (Actual)

March 27, 2026

Last Update Submitted That Met QC Criteria

March 24, 2026

Last Verified

March 1, 2026

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • INSPIRE-PPH-Registry

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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