A Study Investigating Routine Practice of Chronic Thromboembolic Pulmonary Hypertension Management in EMEA Countries

Study Overview
=================
Brief Summary
-----------------
The aim of the registry is the assessment of the diagnosis and treatment of CTEPH (Chronic Thromboembolic Pulmonary Hypertension) in EMEA (Europe/Middle East/Africa) countries.

Official Title
-----------------
EMEA CTEPH Registry: An International Prospective Registry Investigating the Epidemiology, Diagnosis and Treatment of CTEPH Patients in EMEA Countries

Conditions
-----------------
Hypertension, Pulmonary

Intervention / Treatment
-----------------
* Other: Standard of care


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Female and male patients ≥ 18 years of age, diagnosed with CTEPH regardless of the current treatment Availability of a signed informed consent WHO Pulmonary Hypertension clinical classification Group IV (CTEPH): Right heart catheterization (RHC) results that are in line with both of the following haemodynamic levels: Mean pulmonary arterial pressure (PAP) ≥ 25 mmHg at rest Pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg Confirmation of CTEPH diagnosis by one of the following as recommended by standard guidelines: At least 1 (segmental) perfusion defect(s) in ventilation/perfusion (V/Q) scan or Pulmonary artery obstruction seen by MDCT (multidetector computed tomography) angiography or Pulmonary artery obstruction seen by conventional pulmonary cineangiography (In case of suspicion/diagnosis of 'sub-acute' Pulmonary Embolism: Patients who are treated with anti-coagulation for at least 3 months before diagnosis of CTEPH) Exclusion Criteria: Patients with an underlying medical disorder with an anticipated life expectancy less than 6 months Any medical condition which, in the opinion of the investigator, could jeopardize the safety of the patient or his/her compliance in the study, or otherwise make the patient inappropriate for study participation

Ages Eligible for Study
-----------------
Minimum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| CTEPH Patients<br>Patients with confirmed diagnosis of CTEPH | Other: Standard of care<br>* At the discretion of the attending physician.<br>|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| The clinical functional parameters of CTEPH patients will be assessed by evaluating the change in measures of 6MWD between initial and final visit | 6MWD (6 Minutes Walking Distance) | Up to 3 years | 
| The clinical functional parameters of CTEPH patients will be assessed by evaluating the change in measures of WHO Functional Class between initial and final visit | WHO Functional class (World Health Organization Functional Class) | Up to 3 years | 
| The haemodynamics parameters of CTEPH patients will be assessed by evaluating the change in measures of PVR between initial and final visit | PVR (Pulmonary Vascular Resistance) | Up to 3 years | 
| The haemodynamics parameters of CTEPH patients will be assessed by evaluating the change in measures of Cardiac Index (CI) between initial and final visit | CI (Cardiac Index) | Up to 3 years | 
| The haemodynamics parameters of CTEPH patients will be assessed by evaluating the change in measures of mPAP between initial and final visit | mPAP (mean Pulmonary Arterial Pressure) | Up to 3 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Eligibility for PEA | At inclusion, information will be collected for each patient regarding their eligibility for PEA. If eligible, details about the PEA will be recorded; if not eligible, further information will be collected to understand why the patient was not eligible. | Up to 3 years | 
| Rate of recurrent to persistent subtypes after Pulmonary Endarterectomy | Not yet assessable/Recurrent/Residual | Up to 3 years | 
| Time span between onset of symptoms and CTEPH diagnosis |  | Up to 3 years | 
| Usage of diagnostic tools of CTEPH patients (Y/N) |  | Up to 3 years | 
| Rate of Pulmonary Hypertension treatments prior to CTEPH diagnosis, at the time of CTEPH diagnosis and at the time of first visit |  | Up to 3 years | 
| Treatment duration with Pulmonary Hypertension treatments prior to CTEPH diagnosis, at the time of CTEPH diagnosis and at the time of first visit |  | Up to 3 years | 
| Changes in CTEPH treatment during the study period |  | Up to 3 years | 
| Number of patients eligible for Pulmonary endarterectomy |  | Up to 3 years | 
| NT-proBNP (N-terminal Propeptide Brain Natriuretic Peptide) levels |  | Up to 3 years | 
| Mortality due to CTEPH and CTEPH related complications |  | Up to 3 years | 
| Number of healthcare professional visits due to CTEPH and CTEPH related complications |  | Up to 3 years | 
| The total number of days of hospitalization due to CTEPH and CTEPH related complications |  | Up to 3 years | 
| Number of patients eligible for Balloon Pulmonary Angioplasty |  | Up to 3 years | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Type IV Pulmonary Hypertension, Chronic Thromboembolic Pulmonary Hypertension, CTEPH