Chronic thromboembolic pulmonary hypertension
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: Observational study: Patients with CTEPH, TA-PH and FM-PH diagnosed in our hospital from January 1, 2011 to November 2026; Therapeutic research: 1. Diagnosed with fibrosing mediastinitis between November 2024 and November 2026, aged between 18 and 85 years; 2. The patient presented with symptoms of chest tightness, shortness of breath, and reduced exercise tolerance. 3. Chest CT revealed mediastinal lymph node compression of the pulmonary artery, with evidence of pulmonary hypertension consistent with the patient's symptoms. 4. The subject signed the informed consent form prior to participation and is able to comply with the study protocol and one-year follow-up.
Exclusion criteria
Exclusion criteria: Observational study: Patients without complete clinical, imaging, and right heart floating catheter data; Therapeutic research: 1. Currently in the active phase of infection, including but not limited to tuberculosis, Histoplasma capsulatum, and Aspergillus infections; 2. The underlying primary disease, such as sarcoidosis, Behçet’s disease, or uncontrolled IgG4-related disease, is currently not well controlled; 3. There was still a large amount of pleural effusion before the treatment; 4. There are contraindications to bronchoscopy or endovascular intervention; 5. Pulmonary function tests showed FEV1 <30% of the predicted value, FEV1/FVC <30%, and DLCO <30%; 6. Complicated by other end-stage organ dysfunction, such as Child-Pugh class C liver function or stage IV chronic renal failure; 7. Complicated by severe immunosuppression.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Improvement of symptoms and signs;Changes in WHO functional class;Hemodynamic changes;BNP/NT-proBNP levels;6-minute walk distance (6MWD);Changes in pulmonary function;Post-interventional restenosis rate (greater than 50% of the original vessel diameter);Incidence of treatment-related serious adverse events (SAE); | — |
Secondary
| Measure | Time frame |
|---|---|
| Quality of Life score;Changes in cardiac ultrasound parameters;The volume of mediastinal lesions;Clinical deterioration rate (re-hospitalization rate due to right heart failure);Abnormal laboratory or physiological indicators;Complications of bronchoscopy operation and pulmonary vascular interventional therapy; | — |
Countries
China
Contacts
Beijing Chao-Yang Hospital, Capital Medical University