Clinical trial NCT03707561: RUS-registry of PH
Official title: Russian National Registry of Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Pulmonary Hypertension
Lead sponsor: Russian Cardiology Research and Production Center
Sponsor class: OTHER
Collaborators: name: Scientific Research Institute of Rheumatology
First posted: 2018-10-16
Enrollment: 200
Sex: ALL
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
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

Brief summary: 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: 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

Eligibility criteria: 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.

Primary outcomes:
measure: RHC
timeFrame: 12 month
Secondary outcomes:
Other outcomes:
Locations:
Study officials:
affiliation: Russian Cardiology Research and Production Center
name: Tamila Martynyuk, MD, PhD
role: PRINCIPAL_INVESTIGATOR
affiliation: Russian Cardiology Research and Production Center
name: Irina Chazova, MD, PhD
role: STUDY_CHAIR
affiliation: Russian Cardiology Research and Production Center
name: Olga Arkhipova, PhD
role: STUDY_DIRECTOR
affiliation: Institute of Rheumatology named after VA Nasonova, Moscow, Russia
name: Alexander Volkov, MD, PhD.
role: STUDY_DIRECTOR
affiliation: Regional Clinical Hospital №1, Khabarovsk, Russia
name: Vera Lukyantchikova, MD, PhD
role: STUDY_DIRECTOR
affiliation: Belgorod Regional Clinical Hospital, Belgorod, Russia
name: Elena Vetrova, PhD
role: STUDY_DIRECTOR
affiliation: Rostov Regional State CLINICAL HOSPITAL, Russia
name: Elena Devitiyarova, MD, PhD
role: STUDY_DIRECTOR
affiliation: Department of Hospital Therapy of the Voronezh State Medical Academy. N. N. Burdenko, Voronezh, Russia
name: Olga Korolkova, MD, PhD
role: STUDY_DIRECTOR
affiliation: State Healthcare Institution Regional Cardiology Dispensary, Ulyanovsk, Russia
name: Olga Andreyeva
role: STUDY_DIRECTOR
affiliation: Regional Budget Cardiology Dispensary, Astrakhan, Russia
name: Svetlana Martynenko
role: STUDY_DIRECTOR
affiliation: District Cardiology Dispensary "Center for Diagnostic and Cardiovascular Surgery", Surgut, Russia
name: Elena Milovanova, PhD
role: STUDY_DIRECTOR
affiliation: State Future Health Care Institution "Primorskaya Regional Clinical Hospital № 1", Vladivostok, Russia
name: Anna Rogacheva
role: STUDY_DIRECTOR
affiliation: State budgetary health care institution "Bryansk Regional Cardiology Dispensary", Bryansk, Russia
name: Svetlana Kharitonova
role: STUDY_DIRECTOR
affiliation: IVANOVO STATE MEDICAL ACADEMY Department of Therapy and General Practice, Ivanovo, Russia
name: Elena Shutemova, MD, PhD
role: STUDY_DIRECTOR
References:
citation: 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.
citation: 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.
citation: 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.
citation: 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.
citation: 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.