Risk Factor Assessment for Acute Pulmonary Hypertension Crisis

Research on the Clinical Risk Factor Assessment System for Acute Pulmonary Hypertension Crisis and Its Mortality

The goal of this observational study is to learn about pulmonary hypertension crisis (PHC) - a severe, and often fatal complication - in patients with pulmonary hypertension (PH). The main questions this study aims to answer are:

What are the clinical and hemodynamic features of PHC, and what underlying pathophysiological mechanisms cause it to develop? Can these features be used to diagnostic PHC, predict who is at risk of developing it or dying from it, and develop targeted prevention and treatment of PHC?

A multi-center registry platform and biobank will be established to enroll and follow up patients with PH. Clinical data, hemodynamic measurements, and biological specimen will be collected. Risk prediction and early warning tools of PHC will be developed.

Study Overview

• Status: Not yet recruiting
• Conditions: Pulmonary Hypertension; Pulmonary Hypertension Crisis

Detailed Description

Pulmonary hypertension crisis (PHC) is a severe and fatal complication of pulmonary hypertension (PH), characterized by the sudden elevation of pulmonary vascular resistance, acute right hear failure and collapse of systemic circulation. However, PHC currently lacks any internationally accepted definition or diagnostic criteria, and it is still unclear which are the clinical, pathophysiological and molecular risk factors of PHC.

This study aims to establish a large clinical cohort of patients with PH, validate the definition of PHC, identify risk factors of PHC occurrence and mortality, and develop tools for the risk-prediction and early-warning of PHC.

This is a multi-center, prospective cohort study with a retrospective component for model training. Following national health-information standards, a standardized registry dataset will be established to cover demographics, clinical features, laboratory tests, imaging, and hemodynamic profile. Existing PH cohort databases will be integrated and expanded to a target of 5,000 patients. A subset of cases with right heart catheter parameters (RHC) will be extracted. A multi-center biospecimen management platform will be established.

For PHC onset, the investigators will assemble a clinical-molecular-imaging feature set and use convolutional neural networks and tree-based algorithms for feature extraction, then build a cross-modal prediction model using multi-task learning, neural networks, and a Transformer architecture. The model will be prospectively validated and tuned against real-world performance. For PHC mortality, models will be built on patients meeting the new PHC criteria, using 28-day attributable death, all-cause death, and long-term death as endpoints. Models will be trained on a retrospective cohort and then evaluated for accuracy and generalizability on both an internal validation set and an external prospective-cohort validation set.

• Study Type: Observational
• Enrollment (Estimated): 5000

Contacts and Locations

• Study Contact: Name: Run Dong, MD; Phone Number: 861069155036; Email: dongrun5858@foxmail.com

Study Locations

• China
  • Beijing, China, 100730
    • Peking Union Medical College Hospital
    • Contact: Run Dong; Phone Number: +86 10 69155036; Email: dongrun5858@foxmail.com
    • Principal Investigator: Jinmin Peng, MD
  • Shanghai, China
    • Shanghai Pulmonary Hospital
    • Contact: Wenhui Wu, MD; Phone Number: +86 21 65115006; Email: wenhui5621006@126.com
  • Guangdong
    • Guangzhou, Guangdong, China
      • Guangdong Province People's Hospital
      • Contact: Xianmei Li, MD; Phone Number: +86 20 8382 7812; Email: lixianmei@gdph.org.cn
  • Yunnan
    • Kunming, Yunnan, China
      • Yan'an Affiliated Hospital of Kunming Medical University
      • Contact: Hailong Dai, MD; Phone Number: +86 0871 63211157; Email: dhlkm@qq.com

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: N/A

• Sampling Method: Non-Probability Sample

Study Population

Patients with pulmonary hypertension confirmed by right heart catheterization

Description

Inclusion Criteria:

• Pulmonary hypertension confirmed by mean pulmonary arterial pressure (mPAP) over 20 mmHg measured by right heart catheter
• At least once per year of follow-up data collection record

Exclusion Criteria:

• Patients with missing baseline or follow-up data
• Complicated by other diseases with significant influence on survival, such as acute coronary syndrome, malignancy, etc.
• Complicated by other diseases with significant influence on hemodynamics, such as sepsis, acute left heart failure, and acute pulmonary embolism, etc.
• Receiving medications with significant influence on hemodynamics

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
Pulmonary hypertension; Patients with right heart catheter confirmed pulmonary hypertension

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Pulmonary hypertension crisis	Critical increase of pulmonary vascular resistance accompanied by right heart dysfunction and systemic hypoperfusion	From enrollment to 3 years after enrollment
28 day mortality attributed to pulmonary hypertension crisis	Death attributed to pulmonary hypertension crisis within 28 days of hospitalization	From hospitalization to 28 days

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
All cause mortality within hospitalization	All cause death within hospitalization	From hospitalization to discharge or documented death, whichever is earlier, assessed up to 3 years
All cause mortality within 3 years	All cause death within 3 years from the diagnosis of pulmonary hypertension crisis	From the diagnosis of pulmonary hypertension crisis to documented death, assessed up to 3 years

Collaborators and Investigators

• Sponsor: Peking Union Medical College Hospital
• Investigators: Principal Investigator: Jinmin Peng, MD, Peking Union Medical College Hospital

Publications and helpful links

General Publications

• Qian J, Li M, Zhang X, Wang Q, Zhao J, Tian Z, Wei W, Zuo X, Zhang M, Zhu P, Ye S, Zhang W, Zheng Y, Qi W, Li Y, Zhang Z, Ding F, Gu J, Liu Y, Wang Y, Zeng X; following investigators were collaborators in the CSTAR-PAH study:. Long-term prognosis of patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study. Eur Respir J. 2019 Feb 14;53(2):1800081. doi: 10.1183/13993003.00081-2018. Print 2019 Feb.
• Hemnes AR, Beck GJ, Newman JH, Abidov A, Aldred MA, Barnard J, Berman Rosenzweig E, Borlaug BA, Chung WK, Comhair SAA, Erzurum SC, Frantz RP, Gray MP, Grunig G, Hassoun PM, Hill NS, Horn EM, Hu B, Lempel JK, Maron BA, Mathai SC, Olman MA, Rischard FP, Systrom DM, Tang WHW, Waxman AB, Xiao L, Yuan JX, Leopold JA; PVDOMICS Study Group. PVDOMICS: A Multi-Center Study to Improve Understanding of Pulmonary Vascular Disease Through Phenomics. Circ Res. 2017 Oct 27;121(10):1136-1139. doi: 10.1161/CIRCRESAHA.117.311737.
• Qu J, Li M, Zhang X, Zhang M, Zuo X, Zhu P, Ye S, Zhang W, Zheng Y, Qi W, Li Y, Zhang Z, Ding F, Gu J, Liu Y, Qian J, Huang C, Zhao J, Wang Q, Liu Y, Tian Z, Wang Y, Wei W, Zeng X. A prognostic model for systemic lupus erythematosus-associated pulmonary arterial hypertension: CSTAR-PAH cohort study. Respir Res. 2023 Sep 9;24(1):220. doi: 10.1186/s12931-023-02522-2.
• Boucly A, Song S, Keles M, Wang D, Howard LS, Humbert M, Sitbon O, Lawrie A, Thompson AAR, Frank P, Kivimaki M, Rhodes CJ, Wilkins MR. Clustering Patients with Pulmonary Hypertension Using the Plasma Proteome. Am J Respir Crit Care Med. 2025 Aug;211(8):1492-1503. doi: 10.1164/rccm.202408-1574OC.

Study record dates

Study Major Dates

• Study Start (Estimated): July 1, 2026
• Primary Completion (Estimated): June 30, 2029
• Study Completion (Estimated): December 31, 2029

Study Registration Dates

• First Submitted: June 7, 2026
• First Submitted That Met QC Criteria: June 15, 2026
• First Posted (Actual): June 17, 2026

Study Record Updates

• Last Update Posted (Actual): June 17, 2026
• Last Update Submitted That Met QC Criteria: June 15, 2026
• Last Verified: June 1, 2026

More Information

Terms related to this study

• Keywords: Pulmonary hypertension; Diagnosis; Risk factor; Pulmonary hypertension crisis
• Additional Relevant MeSH Terms: Vascular Diseases; Cardiovascular Diseases; Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Hypertension; Pathological Conditions, Signs and Symptoms; Disease; Hypertension, Pulmonary
• Other Study ID Numbers: K7949; 2024ZD0526701 (Other Grant/Funding Number: Ministry of Science and Technology of the People's Republic of China)

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: YES
• IPD Plan Description: IPD that underlie the results reported in our publications will be shared after de-identification, including clinical, hemodynamic, laboratory, and imaging data (tables, figures, and appendices).
• IPD Sharing Time Frame: Beginning 1 year after publication with no end date
• IPD Sharing Access Criteria: Researchers who submit a methodologically sound proposal will be able to access the de-identified individual participant data and supporting documents. Proposals should be directed to the principal investigator at pjm731@hotmail.com. To gain access, requestors will be required to sign a data access agreement, and data will be shared for the purpose of achieving the aims in the approved proposal.
• IPD Sharing Supporting Information Type: STUDY_PROTOCOL; SAP; ICF; ANALYTIC_CODE; CSR

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No