RUS-registry of PH

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

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).

Study Overview

• Status: Unknown
• Conditions: Pulmonary Hypertension
• Intervention / Treatment: Diagnostic test: Right Heart Catheterization (RHC)

Detailed Description

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

• Study Type: Observational
• Enrollment (Anticipated): 200

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

IPAH and heritable PAH, PAH associated with CHD, PAH associated with CTD, portoPH, PAH-HIV, operable CTEPH, non-operable CTEPH

Description

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.

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

7

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Group 1 - IPAH and heritable PAH; Idiopathic pulmonary arterial hypertension (IPAH) and heritable PAH Diagnostic Test: Right Heart Catheterization (RHC)	Diagnostic test: Right Heart Catheterization (RHC); RHC at the enrollment and every 12 months
Group 2- PAH associated with CHD; Pulmonary arterial hypertension associated with congenital heart disease Diagnostic Test: Right Heart Catheterization (RHC)	Diagnostic test: Right Heart Catheterization (RHC); RHC at the enrollment and every 12 months
Group 3- PAH associated with CTD; Pulmonary Arterial Hypertension associated with Connective Tissue Diseases Diagnostic Test: Right Heart Catheterization (RHC)	Diagnostic test: Right Heart Catheterization (RHC); RHC at the enrollment and every 12 months
Group 4- portoPH; Portopulmonary hypertension
group 5- PAH-HIV; Pulmonary arterial hypertension associated with HIV infection
group 6- operable CTEPH; Operable chronic thromboembolic pulmonary hypertension Diagnostic Test: Right Heart Catheterization (RHC)	Diagnostic test: Right Heart Catheterization (RHC); RHC at the enrollment and every 12 months
group 7- non-operable CTEPH; Non operable chronic thromboembolic pulmonary hypertension Diagnostic Test: Right Heart Catheterization (RHC)	Diagnostic test: Right Heart Catheterization (RHC); RHC at the enrollment and every 12 months

What is the study measuring?

Primary Outcome Measures

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

Collaborators and Investigators

• Sponsor: Russian Cardiology Research and Production Center
• Collaborators: Scientific Research Institute of Rheumatology
• Investigators: Principal Investigator: Tamila Martynyuk, MD, PhD, Russian Cardiology Research and Production Center; Study Chair: Irina Chazova, MD, PhD, Russian Cardiology Research and Production Center; Study Director: Olga Arkhipova, PhD, Russian Cardiology Research and Production Center; Study Director: Alexander Volkov, MD, PhD., Institute of Rheumatology named after VA Nasonova, Moscow, Russia; Study Director: Vera Lukyantchikova, MD, PhD, Regional Clinical Hospital №1, Khabarovsk, Russia; Study Director: Elena Vetrova, PhD, Belgorod Regional Clinical Hospital, Belgorod, Russia; Study Director: Elena Devitiyarova, MD, PhD, Rostov Regional State CLINICAL HOSPITAL, Russia; Study Director: Olga Korolkova, MD, PhD, Department of Hospital Therapy of the Voronezh State Medical Academy. N. N. Burdenko, Voronezh, Russia; Study Director: Olga Andreyeva, State Healthcare Institution Regional Cardiology Dispensary, Ulyanovsk, Russia; Study Director: Svetlana Martynenko, Regional Budget Cardiology Dispensary, Astrakhan, Russia; Study Director: Elena Milovanova, PhD, District Cardiology Dispensary "Center for Diagnostic and Cardiovascular Surgery", Surgut, Russia; Study Director: Anna Rogacheva, State Future Health Care Institution "Primorskaya Regional Clinical Hospital № 1", Vladivostok, Russia; Study Director: Svetlana Kharitonova, State budgetary health care institution "Bryansk Regional Cardiology Dispensary", Bryansk, Russia; Study Director: Elena Shutemova, MD, PhD, IVANOVO STATE MEDICAL ACADEMY Department of Therapy and General Practice, Ivanovo, Russia

Publications and helpful links

General Publications

• Chazova IE, Martynyuk TV. [Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1)]. Ter Arkh. 2016;88(9):90-101. doi: 10.17116/terarkh201688990-101. Russian.
• Chazova IE, Martynyuk TV. [Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 2)]. Ter Arkh. 2016;88(10):63-73. doi: 10.17116/terarkh201688663-73. Russian.
• Chazova IE, Avdeev SN, Tsareva NA, Volkov AV, Martyniuk TV, Nakonechnikov SN. [Clinical guidelines for the diagnosis and treatment of pulmonary hypertension]. Ter Arkh. 2014;86(9):4-23. Russian.
• Taran IN, Belevskaya AA, Saidova MA, Martynyuk TV, Chazova IE. Initial Riociguat Monotherapy and Transition from Sildenafil to Riociguat in Patients with Idiopathic Pulmonary Arterial Hypertension: Influence on Right Heart Remodeling and Right Ventricular-Pulmonary Arterial Coupling. Lung. 2018 Dec;196(6):745-753. doi: 10.1007/s00408-018-0160-4. Epub 2018 Sep 4.
• Chazova IE, Arkhipova OA, Valieva ZS, Nakonechnikov SN, Martyniuk TV. [Pulmonary hypertension in Russia: the first results of the national register]. Ter Arkh. 2014;86(9):56-64. Russian.

Helpful Links

• First date of Russian national registry of pulmonary arterial hypertension
• Comparative assessment of the demographic characteristics and functional ability of patients with PAH in the Russian registry and the largest foreign registers.
• Hemodynamic and functional characteristics of patients with different forms of pulmonary arterial hypertension according to the russian registration of patients with pulmonary hypertension.
• First results of a clinical analysis of patients with chronic thromboembolic pulmonary hypertension depending on their operability status according to the data of the Russian pulmonary hypertension register.
• Features of pulmonary arterial hypertension according to the Russian Register.

Study record dates

Study Major Dates

• Study Start: January 1, 2016
• Primary Completion (Anticipated): December 1, 2020
• Study Completion (Anticipated): January 1, 2021

Study Registration Dates

• First Submitted: October 11, 2018
• First Submitted That Met QC Criteria: October 11, 2018
• First Posted (Actual): October 16, 2018

Study Record Updates

• Last Update Posted (Actual): October 25, 2018
• Last Update Submitted That Met QC Criteria: October 23, 2018
• Last Verified: October 1, 2018

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hypertension; Hypertension, Pulmonary
• Other Study ID Numbers: NMIC for Cardiology

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No