Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry

Aim. To assess demographic and clinical characteristics, to describe of the functional and hemodynamic status, profile of concomitant pathology, data of instrumental examination in chronic thromboembolic pulmonary hypertension (CTEPH) patients; to study the features of specific and supportive therap...

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Autores principales:	Zarina S. Valieva, Tamila V. Martynyuk, Sergei N. Nakonechnikov, Irina E. Chazova
Formato:	article
Lenguaje:	RU
Publicado:	"Consilium Medicum" Publishing house 2021
Materias:	chronic thromboembolic pulmonary hypertension specific drug therapy russian national registry Medicine R
Acceso en línea:	https://doaj.org/article/8502614469424b34a55becaf06eeb2dd
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spelling	oai:doaj.org-article:8502614469424b34a55becaf06eeb2dd2021-12-01T12:22:36ZCharacteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry0040-36602309-534210.26442/00403660.2021.09.201037https://doaj.org/article/8502614469424b34a55becaf06eeb2dd2021-09-01T00:00:00Zhttps://ter-arkhiv.ru/0040-3660/article/viewFile/82971/63450https://doaj.org/toc/0040-3660https://doaj.org/toc/2309-5342Aim. To assess demographic and clinical characteristics, to describe of the functional and hemodynamic status, profile of concomitant pathology, data of instrumental examination in chronic thromboembolic pulmonary hypertension (CTEPH) patients; to study the features of specific and supportive therapy according to the data of the Russian national registry. Materials and methods. From 2012 till 2020 a multicenter, prospective study in 15 regional expert centers of the Russian Federation (www.medibase.pro) included 404 newly diagnosed CTEPH patients over the age of 18 years in the Russian registry of patients with pulmonary arterial hypertension and CTEPH (NCT03707561). The diagnosis was established by European and Russian clinical guidelines for the diagnosis and management of pulmonary hypertension. 154 inoperable CTEPH patients an additional analysis of specific and supportive therapy was performed. Results. The study included 404 patients (55.6% women and 44.3% men) at the age of 58.6 [48.6; 69.3] years. Median time from symptom onset to the diagnosis verification waswas 2.4 years (from 0.1 to 2.9 years). 79.1% of patients were in the III and IV functional class (World Health Organization) at the time of diagnosis and in 44.1% with RHF (right heart failure). In assessing the profile of concomitant pathology, it was noted that CTEPH patients were more often with arterial hypertension (39.1%), erosive-ulcerative lesions of the stomach/duodenum (16.1%), atrial fibrillation (13.8%), obesity (13.1%). Distance in 6MWD (6-min walk distance) was 337.2 [250; 422] m, Borg dyspnea index scale 4.1 [3.0; 5.0] points. Hemodynamic parameters according to right heart catheterization were: Mean PAP (pulmonary arterial pressure) (51.114.04) mmHg, CO (cardiac output) (3.50.98) l/min, CI (cardiac index) (2.00.48) l/min/m2, PVR (2008528) dyns/cm5. Conclusion. According to the Russian registry, inoperable CTEPH patients had precapillary PH (pulmonary hypertension) with severe functional status, in combination with frequent concomitant pathology (arterial hypertension, erosive-ulcerative lesions of the stomach/duodenum, atrial fibrillation, obesity, right heart failure). 66% of inoperable CTEPH patients received specific drug therapy.Zarina S. ValievaTamila V. MartynyukSergei N. NakonechnikovIrina E. Chazova"Consilium Medicum" Publishing housearticlechronic thromboembolic pulmonary hypertensionspecific drug therapyrussian national registryMedicineRRUТерапевтический архив, Vol 93, Iss 9, Pp 1058-1065 (2021)
institution	DOAJ
collection	DOAJ
language	RU
topic	chronic thromboembolic pulmonary hypertension specific drug therapy russian national registry Medicine R
spellingShingle	chronic thromboembolic pulmonary hypertension specific drug therapy russian national registry Medicine R Zarina S. Valieva Tamila V. Martynyuk Sergei N. Nakonechnikov Irina E. Chazova Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry
description	Aim. To assess demographic and clinical characteristics, to describe of the functional and hemodynamic status, profile of concomitant pathology, data of instrumental examination in chronic thromboembolic pulmonary hypertension (CTEPH) patients; to study the features of specific and supportive therapy according to the data of the Russian national registry. Materials and methods. From 2012 till 2020 a multicenter, prospective study in 15 regional expert centers of the Russian Federation (www.medibase.pro) included 404 newly diagnosed CTEPH patients over the age of 18 years in the Russian registry of patients with pulmonary arterial hypertension and CTEPH (NCT03707561). The diagnosis was established by European and Russian clinical guidelines for the diagnosis and management of pulmonary hypertension. 154 inoperable CTEPH patients an additional analysis of specific and supportive therapy was performed. Results. The study included 404 patients (55.6% women and 44.3% men) at the age of 58.6 [48.6; 69.3] years. Median time from symptom onset to the diagnosis verification waswas 2.4 years (from 0.1 to 2.9 years). 79.1% of patients were in the III and IV functional class (World Health Organization) at the time of diagnosis and in 44.1% with RHF (right heart failure). In assessing the profile of concomitant pathology, it was noted that CTEPH patients were more often with arterial hypertension (39.1%), erosive-ulcerative lesions of the stomach/duodenum (16.1%), atrial fibrillation (13.8%), obesity (13.1%). Distance in 6MWD (6-min walk distance) was 337.2 [250; 422] m, Borg dyspnea index scale 4.1 [3.0; 5.0] points. Hemodynamic parameters according to right heart catheterization were: Mean PAP (pulmonary arterial pressure) (51.114.04) mmHg, CO (cardiac output) (3.50.98) l/min, CI (cardiac index) (2.00.48) l/min/m2, PVR (2008528) dyns/cm5. Conclusion. According to the Russian registry, inoperable CTEPH patients had precapillary PH (pulmonary hypertension) with severe functional status, in combination with frequent concomitant pathology (arterial hypertension, erosive-ulcerative lesions of the stomach/duodenum, atrial fibrillation, obesity, right heart failure). 66% of inoperable CTEPH patients received specific drug therapy.
format	article
author	Zarina S. Valieva Tamila V. Martynyuk Sergei N. Nakonechnikov Irina E. Chazova
author_facet	Zarina S. Valieva Tamila V. Martynyuk Sergei N. Nakonechnikov Irina E. Chazova
author_sort	Zarina S. Valieva
title	Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry
title_short	Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry
title_full	Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry
title_fullStr	Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry
title_full_unstemmed	Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry
title_sort	characteristics of patients with chronic thromboembolic pulmonary hypertension according to the russian national registry
publisher	"Consilium Medicum" Publishing house
publishDate	2021
url	https://doaj.org/article/8502614469424b34a55becaf06eeb2dd
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Characteristics of patients with chronic thromboembolic pulmonary hypertension according to the Russian National Registry

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