Resumen

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.

Elegibilidad

Edad mínima: 18 YearsSexo: ALL

Criterios de inclusión

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

Criterios de exclusión

Main cause of PH other than CTEPH
Participating in an interventional clinical trial at enrollment

Intervenciones

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

TrEatment Approach in the Multimodal Era Registry