Chronic Thromboembolic Disease Registry

Study Overview
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Brief Summary
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The goal of this patient registry is to learn about the natural history in patients with chronic thromboembolic disease (CTD) and/or chronic thromboembolic pulmonary hypertension (CTEPH). The main question[s] it aims to answer are: Long-term outcome after various types of treatment, including medication, balloon pulmonary angioplasty, and pulmonary endarterectomy Effect of treatment on patient's quality of life and exercise tolerance Participants will be followed longitudinally to assess their health outcomes and quality of life via chart review and health quality surveys.

Official Title
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Chronic Thromboembolic Disease: A Prospective Registry

Conditions
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Chronic Thromboembolic Pulmonary Hypertension, Chronic Thromboembolic Disease

Intervention / Treatment
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* Other: Survey


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients referred with CTED and/or CTEPH defined as: 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 chronic thromboembolic pulmonary disease (CTEPD) without pulmonary hypertension (PH) Radiologic finding of chronic thromboembolic disease, including abnormal ventilation perfusion scan, pulmonary angiogram, computer tomographic pulmonary angiogram, or magnetic resonance pulmonary angiogram Post-embolic exercise intolerance and evidence of chronic thromboembolic disease in the absence of resting pulmonary hypertension Treatment with anticoagulation for ≥ 3 months before diagnosis of CTEPH or CTEPD without PH Age ≥ 18 years Exclusion Criteria: Main cause of PH other than World Health Organization (WHO) group 4 (CTEPH) Patient's refusal to participate in clinical research and/or receive intervention

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

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Chronic thromboembolic disease<br>Patients with chronic thromboembolic disease/pulmonary hypertension | Other: Survey<br>* Patients who received medical, surgical or interventional treatment for chronic thromboembolic pulmonary hypertension will be followed and given quality of life questionnaires.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Total number of patients who survive | The total number of patients who are alive | Through study completion, an average of 5 years | 
| Changes in exercise tolerance | Measured by 6-min walk test, which will be done approximately every 3 months at follow up per standard of care | Baseline to end of the study, an average of 5 years | 
| Changes in supplemental oxygen use severity | Number of patients on supplemental oxygen, which will be recorded approximately every 3 months at follow up per standard of care. This will be compiled at the end of the study to provide overall change in measurement. | Baseline to end of the study, an average of 5 years | 
| Changes in New York Heart Association (NYHA) functional class | Grading of patients by NYHA functional class, which include functional capacity and objective assessment. This will be compiled at the end of the study to provide overall change in measurement. | Baseline to end of the study, an average of 5 years | 
| European Quality of Life Five Dimension (EQ-5D) - Scale Score | Assessment of quality of life will be measured using EQ-5D-5L. This instrument is a self-assessed, health-related, quality of life questionnaire that measures quality of life on a 5-component scale. The scores range from Level 1 to Level 5, with lower scores indicating a higher quality of life. This will be collected every 3 months and compiled at the end of the study to provide overall change in measurement. | Baseline to end of the study, an average of 5 years | 
| Changes in patients' emPHasis-10 scores | Assessed by the emPHasis-10 (pulmonary hypertension) instrument, which is an instrument specifically designed to assess the quality of life in patients with pulmonary hypertension. Each item will be scored from 0 to 5 where 0 was the best score. This will be collected every 3 months and compiled at the end of the study to assess overall change in measurement. | Baseline to end of the study, an average of 5 years | 
| Number of patients undergoing lung transplantation | Number of patients undergoing lung transplantation | Baseline to end of the study, an average of 5 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Changes in CTEPH-/CTED- specific medication | The frequency and dose of medication specifically for management of CTEPH or CTED, which will be assessed approximately every 3 months as routine medical follow up. This will be compiled at the end of the study to assess overall change. | Baseline to end of the study, an average of 5 years | 
| History of undergoing pulmonary endarterectomy | Number of patients undergoing pulmonary endarterectomy | Through study completion, an average of 5 years | 
| History of undergoing pulmonary balloon angioplasty | Number of patients undergoing pulmonary balloon angioplasty | Through study completion, an average of 5 years |