RUS-registry of PH

Study Overview
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Brief Summary
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Russian National Registry of Patients With Pulmonary Arterial Hypertension (PAH) and Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a multicenter, observational study of the clinical course and disease management of PAH and CTEPH patients. designed to gather demographic, clinical and prognostic data of routine medical care in prospective manner for newly initiated treatment since Jan 2016. The internet-based registry (www.medibase.pro) fulfills high quality standards through several measures (planned minimum centre contribution of at least 10 patients per year, automated plausibility checks of data at entry, queries, monitoring with source data verification in >50% of participating centers). All consecutive patients diagnosed with World Health Organization Pulmonary Hypertension Groups (WHO Group I) PAH according to specific hemodynamic criteria will be enrolled in participating centers after signing the informed consents. Participating patients will be followed for a minimum of five years from the time of enrollment. It can be applied, among further purposes, for quality assurance: individual centers can confidentially compare their results with the combined outcome of the other centers. It is expected that the registry contributes to optimization of specific drug therapy for PAH and Pulmonary Hypertension (PH).

Detailed Description
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Russian National Registry of Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension (RUS-registry of PH) will report current and comprehensive data on Demographics, clinical course of incident PAH and CTEPH, the hemodynamic and functional status of patients The main diagnostic approaches for the diagnosis of PAH and CTEPH. Patient outcomes including survival, by subgroup, by treatment strategy and other factors Clinical predictors of short-term and long-term clinical outcomes Relationship between PAH- treatment medications and patient outcomes Temporal trends in treatments and outcomes for newly diagnosed patients The state of implementation of current PAH guidelines To describe the status of operable or non-operable patients with PAH and CTEPH To identify the most important prognostic indicators in patients with PH. To study the basic principles of therapy of patients with PH.patients To evolve research needs of the PAH community

Official Title
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Russian National Registry of Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

Conditions
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Pulmonary Hypertension

Intervention / Treatment
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* Diagnostic Test: Right Heart Catheterization (RHC)


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Newly diagnosed PAH: idiopathic form (IPAH) or PAH associated with connective tissue diseases (PAH-CTD), with congenital heart defects (PAH-CHD), with HIV infection (PAH-HIV), or portopulmonary hypertension Newly diagnosed CTEPH- operable and non-operable Documentation of the following hemodynamic parameters by right heart catheterization, performed at the time of study enrollment: Mean pulmonary arterial pressure (mPAP) ≥ 25 mm Hg at rest Pulmonary wedge pressure < 15 mm Hg Pulmonary vascular resistance (PVR) ≥ 240 dynes.sec.cm-5 (i.e., ≥ 3.0 Wood units) Signed informed consent Previously naïve patients with newly initiated therapy with endothelin receptor antagonists (ERA), phoshodiesterase-5 (PDE-5) inhibitors, soluble guanylate cyclase (sGC) stimulators or prostacyclins in mono- or combination therapy.
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 |
| --- | --- |
| Group 1 - IPAH and heritable PAH<br>Idiopathic pulmonary arterial hypertension (IPAH) and heritable PAH Diagnostic Test: Right Heart Catheterization (RHC) | Diagnostic Test: Right Heart Catheterization (RHC)<br>* RHC at the enrollment and every 12 months<br>|
| Group 2- PAH associated with CHD<br>Pulmonary arterial hypertension associated with congenital heart disease Diagnostic Test: Right Heart Catheterization (RHC) | Diagnostic Test: Right Heart Catheterization (RHC)<br>* RHC at the enrollment and every 12 months<br>|
| Group 3- PAH associated with CTD<br>Pulmonary Arterial Hypertension associated with Connective Tissue Diseases Diagnostic Test: Right Heart Catheterization (RHC) | Diagnostic Test: Right Heart Catheterization (RHC)<br>* RHC at the enrollment and every 12 months<br>|
| Group 4- portoPH<br>Portopulmonary hypertension | |
| group 5- PAH-HIV<br>Pulmonary arterial hypertension associated with HIV infection | |
| group 6- operable CTEPH<br>Operable chronic thromboembolic pulmonary hypertension Diagnostic Test: Right Heart Catheterization (RHC) | Diagnostic Test: Right Heart Catheterization (RHC)<br>* RHC at the enrollment and every 12 months<br>|
| group 7- non-operable CTEPH<br>Non operable chronic thromboembolic pulmonary hypertension Diagnostic Test: Right Heart Catheterization (RHC) | Diagnostic Test: Right Heart Catheterization (RHC)<br>* RHC at the enrollment and every 12 months<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| RHC | Change of pulmonary vascular resistance | 12 month |