TrEatment Approach in the Multimodal Era Registry

Study Overview
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Brief Summary
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The goal of this observational patient registry is to learn how expert centers treat patients with chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH is a condition in which blood clots block the blood vessels in the lungs. There are currently three treatment options for patients with CTEPH: surgery to remove blood clots from large vessels in the lungs (pulmonary endarterectomy (PEA)) the use of a small balloon to unblock smaller blood vessels (balloon pulmonary angioplasty (BPA)) drugs Patients can also receive a combination of these treatments. The main question this registry aims to answer are: How many patients receive a given kind of treatment? How do expert centers combine the different treatments? Are patients doing better after they receive a given kind of treatment? How many patients are alive 1, 3 and 5 years after they receive a given kind of treatment? Participants will receive the same treatments that they would receive if they did not participate in the study. During the study, patients will visit their doctors as they would do normally. The doctors will collect information on the patients' health and enter it into the study database. The follow-up time will be at least 3 years for all patients.

Official Title
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TrEatment Approach in the Multimodal Era Registry

Conditions
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Chronic Thromboembolic Pulmonary Hypertension, CTEPH

Intervention / Treatment
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* Procedure: Pulmonary endarterectomy
* Procedure: Balloon pulmonary angioplasty
* Drug: Pulmonary hypertension (PH)-specific medication


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Newly referred patient with CTEPH or chronic thromboembolic pulmonary disease (CTEPD) without PH according to the following criteria: Mean pulmonary arterial pressure (mPAP) > 20 mmHg at rest with pulmonary vascular resistance (PVR) > 2 Wood units (WU); or if mPAP ≤ 20 mmHg or PVR ≤ 2 WU at rest, have exercise limitations from CTEPD without PH Abnormal ventilation perfusion scan, pulmonary angiogram, computer tomographic pulmonary angiogram, or magnetic resonance pulmonary angiogram confirming CTEPD as recommended by standard guidelines Treatment with anticoagulation for ≥ 3 months before diagnosis of CTEPH or CTEPD without PH Naïve to interventional treatment (both PEA and BPA) Pre-treatment with PH-specific medication for ≤ 12 months, or no medical pre-treatment, at enrollment Willing and able to provide informed consent in order to participate in the study (informed consent signed) Age ≥ 18 years CTEPH-specific treatment must be modified or initiated at the participating site Exclusion Criteria: Main cause of PH other than CTEPH Participating in an interventional clinical trial at enrollment

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Procedure: Pulmonary endarterectomy|Surgical removal of a chronic clot from the pulmonary artery|
|Procedure: Balloon pulmonary angioplasty|Catheter-based intervention to dilate occluded pulmonary vessels through the insertion and inflation of a small balloon|
|Drug: Pulmonary hypertension (PH)-specific medication|Treatment with any PH-specific medication, i.e. endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, drugs acting on the prostanoid pathway and prostaglandin I2 receptor agonists|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Utilization and combination of treatment modalities | Proportion of patients undergoing a certain type of treatment modality, or a certain combination of modalities | Min. 3 years | 
| 3-year survival | Proportion of patients alive 3 years after the last intervention recorded in the registry, or after start of medical treatment for patients not undergoing any interventional treatment | 3 years after last intervention | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Complications of treatment interventions (per patient) | Proportion of patients experiencing complications from BPA or PEA | Min. 3 years | 
| Complications of treatment interventions (per BPA session) | Proportion of BPA sessions associated with complications | Min. 3 years | 
| 1-year survival | Proportion of patients alive 1 year after the last intervention recorded in the registry, or after start of medical treatment for patients not undergoing any interventional treatment | 1 year after last intervention | 
| 5-year survival | Proportion of patients alive 5 years after the last intervention recorded in the registry, or after start of medical treatment for patients not undergoing any interventional treatment | 5 year after last intervention | 
| Changes in PH-specific medication | Proportion of patients experiencing a change in PH-specific medication during the study | Min. 3 years | 
| Reason why PEA was not performed | The main reason why PEA was not performed as chosen by the investigator from a list of pre-defined reasons | Min. 3 years | 
| Reason why BPA was not performed | The main reason why BPA was not performed as chosen by the investigator from a list of pre-defined reasons | Min. 3 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Chronic thromboembolic pulmonary hypertension, CTEPH, Pulmonary endarterectomy, PEA, Balloon pulmonary angioplasty, BPA, Multimodal treatment