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Characteristics and survival of adult Swedish PAH and CTEPH patients 2000–2014

Rådegran, Göran LU ; Kjellström, Barbro; Ekmehag, Björn LU ; Larsen, Flemming; Rundqvist, Bengt; Blomquist, Sofia Berg; Gustafsson, Carola; Hesselstrand, Roger LU ; Karlsson, Monica LU and Kornhall, Björn LU , et al. (2016) In Scandinavian Cardiovascular Journal 50(4). p.243-250
Abstract

Objectives: 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. Design: Incident PAH and CTEPH patients 2008–2014 from all seven Swedish PAH-centres were specifically reviewed. Results: 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... (More)

Objectives: 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. Design: Incident PAH and CTEPH patients 2008–2014 from all seven Swedish PAH-centres were specifically reviewed. Results: 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. Conclusion: 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.

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publication status
published
subject
keywords
Incident, prevalent, pulmonary hypertension, survival
in
Scandinavian Cardiovascular Journal
volume
50
issue
4
pages
243 - 250
publisher
Taylor & Francis
external identifiers
  • scopus:84974779285
ISSN
1401-7431
DOI
10.1080/14017431.2016.1185532
language
English
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yes
id
c1a1aec5-98c2-415a-94f6-7df649c190fa
date added to LUP
2016-07-11 20:02:49
date last changed
2017-03-13 12:13:33
@article{c1a1aec5-98c2-415a-94f6-7df649c190fa,
  abstract     = {<p>Objectives: 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. Design: Incident PAH and CTEPH patients 2008–2014 from all seven Swedish PAH-centres were specifically reviewed. Results: 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 &gt;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. Conclusion: The majority of the PAH and CTEPH patients were diagnosed at age &gt;65 years, in functional class III–IV, and exhibiting several comorbidities. PAH survival in SPAHR was similar to other registers.</p>},
  author       = {Rådegran, Göran and Kjellström, Barbro and Ekmehag, Björn and Larsen, Flemming and Rundqvist, Bengt and Blomquist, Sofia Berg and Gustafsson, Carola and Hesselstrand, Roger and Karlsson, Monica and Kornhall, Björn and Nisell, Magnus and Persson, Liselotte and Ryftenius, Henrik and Selin, Maria and Ullman, Bengt and Wall, Kent and Wikström, Gerhard and Willehadson, Maria and Jansson, Kjell},
  issn         = {1401-7431},
  keyword      = {Incident,prevalent,pulmonary hypertension,survival},
  language     = {eng},
  month        = {07},
  number       = {4},
  pages        = {243--250},
  publisher    = {Taylor & Francis},
  series       = {Scandinavian Cardiovascular Journal},
  title        = {Characteristics and survival of adult Swedish PAH and CTEPH patients 2000–2014},
  url          = {http://dx.doi.org/10.1080/14017431.2016.1185532},
  volume       = {50},
  year         = {2016},
}