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Characteristics and survival of adult Swedish PAH and CTEPH patients 2000-2014
Department of Clinical Sciences Lund, Cardiology, Lund University, Lund, Sweden; Haemodynamic Laboratory, the Section for Heart Failure and Valvular Disease, VO Heart and Lung Medicine, Skåne University Hospital, Lund, Sweden.
Cardiology Unit, Department of Medicine, Karolinska Institute, Stockholm, Sweden.
Department of Public Health and Caring Science, Uppsala University and Uppsala University Hospital, Uppsala, Sweden.
Department of Molecular Medicine and Surgery, Section of Clinical Physiology, Karolinska Institute, Stockholm, Sweden; Department of Clinical Physiology, Karolinska University Hospital, Stockholm, Sweden.
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2016 (English)In: Scandinavian Cardiovascular Journal, ISSN 1401-7431, E-ISSN 1651-2006, Vol. 50, no 4, 243-250 p.Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
Taylor & Francis, 2016. Vol. 50, no 4, 243-250 p.
Keyword [en]
Incident, prevalent, pulmonary hypertension, survival
National Category
Clinical Medicine
URN: urn:nbn:se:liu:diva-130206DOI: 10.1080/14017431.2016.1185532ISI: 000379819900008PubMedID: 27146648OAI: diva2:948978

Funding agencies:  Actelion Pharmaceuticals Sweden AB; Bayer Health Care; Eli Lilly Sweden; Glaxo-SmithKline; Nordicinfu Care; Pfizer; SALAR (SKL); "ALF" foundations

Available from: 2016-07-14 Created: 2016-07-14 Last updated: 2016-08-19Bibliographically approved

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Karlsson, MonicaJansson, Kjell
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Department of Cardiology in LinköpingDepartment of Clinical Physiology in LinköpingDivision of Cardiovascular MedicineFaculty of Medicine and Health Sciences
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Scandinavian Cardiovascular Journal
Clinical Medicine

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