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Association of Right Atrial Mechanics with Hemodynamics and Physical Capacity in Patients with Idiopathic Pulmonary Arterial Hypertension: Insight from a Single-Center Cohort in Northern Sweden.

https://arctichealth.org/en/permalink/ahliterature277234
Source
Echocardiography. 2016 Jan;33(1):46-56
Publication Type
Article
Date
Jan-2016
Author
Samir Kanti Saha
Stefan Söderberg
Per Lindqvist
Source
Echocardiography. 2016 Jan;33(1):46-56
Date
Jan-2016
Language
English
Publication Type
Article
Keywords
Cohort Studies
Exercise Test
Exercise Tolerance - physiology
Familial Primary Pulmonary Hypertension - physiopathology
Female
Heart Atria - physiopathology - ultrasonography
Heart Diseases - physiopathology - ultrasonography
Hemodynamics - physiology
Humans
Male
Middle Aged
Sweden
Abstract
Role of right atrial mechanics has not been studied in idiopathic pulmonary arterial hypertension (IPAH). We sought to investigate the correlation between the right sided, particularly right atrial mechanics, hemodynamic parameters, and functional capacity prior to initiation of treatment.
Twenty-five subjects (63 ± 17 years, 13 males) with newly diagnosed IPAH by right heart catheterization (RHC) underwent speckle tracking echocardiography (STE) of the right ventricle (RV) and right atrium (RA). All the patients had a six-minute walk test (6MWD) performed, functional class (WHO-FC) assessed, and plasma NT-proBNP estimated. STE strain imaging of RA and RV was performed offline. STE of the RA myocardium yielded characteristic strain (S%) and strain rate (SR) curves with clear delineation of its reservoir (RA-RS%), conduit (RA-SR E), and booster pump functions (RA-SR A). In IPAH, RHC-obtained pulmonary vascular resistance (PVR) and pulmonary artery systolic pressure (PASP), respectively, were 7.3 ± 5.6 Wood units and 66 ± 24 mmHg. RA-RS% was associated negatively with PVR (r = -0.46; P
PubMed ID
26096217 View in PubMed
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Characteristics and survival of adult Swedish PAH and CTEPH patients 2000-2014.

https://arctichealth.org/en/permalink/ahliterature281202
Source
Scand Cardiovasc J. 2016 Aug;50(4):243-50
Publication Type
Article
Date
Aug-2016
Author
Göran Rådegran
Barbro Kjellström
Björn Ekmehag
Flemming Larsen
Bengt Rundqvist
Sofia Berg Blomquist
Carola Gustafsson
Roger Hesselstrand
Monica Karlsson
Björn Kornhall
Magnus Nisell
Liselotte Persson
Henrik Ryftenius
Maria Selin
Bengt Ullman
Kent Wall
Gerhard Wikström
Maria Willehadson
Kjell Jansson
Source
Scand Cardiovasc J. 2016 Aug;50(4):243-50
Date
Aug-2016
Language
English
Publication Type
Article
Keywords
Aged
Comorbidity
Familial Primary Pulmonary Hypertension - diagnosis - epidemiology
Female
Humans
Hypertension, Pulmonary - diagnosis - epidemiology - etiology
Incidence
Male
Patient Acuity
Pulmonary Embolism - complications
Survival Analysis
Sweden - epidemiology
Abstract
The Swedish Pulmonary Arterial Hypertension Register (SPAHR) is an open continuous register, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients from 2000 and onwards. We hereby launch the first data from SPAHR, defining baseline characteristics and survival of Swedish PAH and CTEPH patients.
Incident PAH and CTEPH patients 2008-2014 from all seven Swedish PAH-centres were specifically reviewed.
There were 457 PAH (median age: 67 years, 64% female) and 183 CTEPH (median age: 70 years, 50% female) patients, whereof 77 and 81%, respectively, were in functional class III-IV at diagnosis. Systemic hypertension, diabetes, ischaemic heart disease and atrial fibrillation were common comorbidities, particularly in those >65 years. One-, 3- and 5-year survival was 85%, 71% and 59% for PAH patients. Corresponding numbers for CTEPH patients with versus without pulmonary endarterectomy were 96%, 89% and 86% versus 91%, 75% and 69%, respectively. In 2014, the incidence of IPAH/HPAH, associated PAH and CTEPH was 5, 3 and 2 per million inhabitants and year, and the prevalence was 25, 24 and 19 per million inhabitants.
The majority of the PAH and CTEPH patients were diagnosed at age >65 years, in functional class III-IV, and exhibiting several comorbidities. PAH survival in SPAHR was similar to other registers.
PubMed ID
27146648 View in PubMed
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Information, social support and coping in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension-A nationwide population-based study.

https://arctichealth.org/en/permalink/ahliterature291337
Source
Patient Educ Couns. 2017 May; 100(5):936-942
Publication Type
Journal Article
Date
May-2017
Author
Bodil Ivarsson
Göran Rådegran
Roger Hesselstrand
Barbro Kjellström
Author Affiliation
Lund University, Department of Clinical Sciences, Lund, Cardiothoracic Surgery, and Skåne University Hospital, and Medicine Services University Trust, Region Skåne, Lund, Sweden. Electronic address: bodil.ivarsson@med.lu.se.
Source
Patient Educ Couns. 2017 May; 100(5):936-942
Date
May-2017
Language
English
Publication Type
Journal Article
Keywords
Adaptation, Psychological
Adult
Aged
Aged, 80 and over
Cohort Studies
Cross-Sectional Studies
Familial Primary Pulmonary Hypertension - psychology
Female
Health Knowledge, Attitudes, Practice
Humans
Hypertension, Pulmonary - psychology
Male
Middle Aged
Pain Management
Population Surveillance
Pulmonary Embolism
Social Support
Surveys and Questionnaires
Sweden
Abstract
Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) decrease life expectancy for affected patients. The aim was to describe the perceptions of received information regarding diagnosis, treatment and management, as well as social support and coping perceived by patients with PAH or CTEPH.
A descriptive, national cohort survey that included patients from all PAH-centres in Sweden. A quantitative methodology was employed where all patients received three questionnaires, QLQ-INFO25, Mastery Scale and Social Network & Support Scale.
The response rate was 74% (n=325), mean age 66±14years, 71% were female and 69% were diagnosed with PAH and 31% with CTEPH. Sixty percent of respondents were satisfied with the received information and felt it was helpful. Best experiences were of information in the areas of medical tests (70%) and the disease (61%). Forty-five percent wanted more information. Men described their social network as more accessible than women (p=0.004). Those living alone had a lower coping ability.
Despite a high level of satisfaction with the received information, almost half of the patients wanted more information.
Regular repeating or giving new information should be an ongoing process in the care of patients at the PAH-centres.
PubMed ID
28041647 View in PubMed
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Prognosis and response to first-line single and combination therapy in pulmonary arterial hypertension.

https://arctichealth.org/en/permalink/ahliterature261365
Source
Scand Cardiovasc J. 2014 Aug;48(4):223-33
Publication Type
Article
Date
Aug-2014
Author
David Kylhammar
Liselotte Persson
Roger Hesselstrand
Göran Rådegran
Source
Scand Cardiovasc J. 2014 Aug;48(4):223-33
Date
Aug-2014
Language
English
Publication Type
Article
Keywords
Adult
Aged
Antihypertensive Agents - therapeutic use
Arterial Pressure - drug effects
Atrial Function, Right - drug effects
Atrial Pressure - drug effects
Drug Therapy, Combination
Exercise Tolerance - drug effects
Familial Primary Pulmonary Hypertension - drug therapy - physiopathology
Female
Genetic Predisposition to Disease
Humans
Hypertension, Pulmonary - diagnosis - drug therapy - genetics - mortality - physiopathology
Kaplan-Meier Estimate
Male
Middle Aged
Proportional Hazards Models
Pulmonary Artery - drug effects - physiopathology
Recovery of Function
Retrospective Studies
Sweden
Time Factors
Treatment Outcome
Vascular Resistance - drug effects
Abstract
To investigate survival, treatment escalation, effects of first-line single- and first-line combination therapy and prognostic markers in idiopathic- (IPAH), hereditary- (HPAH) and connective tissue disease-associated (CTD-PAH) pulmonary arterial hypertension (PAH).
Retrospective analysis of medical journals from PAH patients at Skåne University Hospital 2000-2011.
1-, 2- and 3-year survival was 87%, 67%, and 54%, respectively, for the entire population, but worse (p = 0.003) in CTD-PAH than IPAH/HPAH. After 1, 2 and 3 years, 58%, 41% and 24% of patients starting on single therapy were alive on single therapy. 37.5% of patients on first-line single therapy received escalated treatment at first follow-up. First-line combination therapy more greatly decreased pulmonary vascular resistance index (PVRI, p = 0.017) than first-line single therapy. Only first-line combination therapy improved (p = 0.042) cardiac index (CI). Higher mean right atrial pressure (MRAP, p = 0.018), MRAP/CI (p = 0.021) and WHO functional class (p
PubMed ID
24912571 View in PubMed
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[Socio-economic burden of pulmonary hypertension: relevance of assessment in Russia and the world].

https://arctichealth.org/en/permalink/ahliterature305439
Source
Ter Arkh. 2020 Apr 27; 92(3):125-131
Publication Type
Journal Article
Review
Date
Apr-27-2020
Author
V D Zakiev
A D Gvozdeva
T V Martynyuk
Author Affiliation
Myasnikov Institute of Clinical Cardiology, National Medical Research Center of Cardiology.
Source
Ter Arkh. 2020 Apr 27; 92(3):125-131
Date
Apr-27-2020
Language
Russian
Publication Type
Journal Article
Review
Keywords
Cost of Illness
Familial Primary Pulmonary Hypertension
Humans
Hypertension, Pulmonary
Quality of Life
Russia
Abstract
Pulmonary hypertension (PH) is a progressive disease which is characterized with the increase of pulmonary artery pressure and pulmonary vascular resistance. Such condition leads to right ventricular heart failure and premature death of patients. Pulmonary arterial hypertension (PAH) has the status of an orphan disease. However in Russia only idiopathic PH is included in the list of 24 life-threatening and chronic progressive rare diseases, while other forms of PH are not in it. Inclusion in this list guarantees drug provision for patients at the expense of the regional budget, while patients with other forms of PH can rely on free medication only if they have a disability. The lack of criteria for revising this list as well as the imperfection of legal regulation in the field of drug support for orphan diseases leads to high disability, a significant decrease in the duration and quality of life of patients with PH. As part of a multicriteria approach, a clinical and economic analysis of the disease burden can be one of the tools for policy development and decision-making on the distribution of funding in the healthcare. The article provides a review of the economic burden of various forms of PH in the world.
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PubMed ID
32598804 View in PubMed
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