title: Characteristics and survival of adult Swedish PAH and CTEPH patients 2000-2014.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: a Department of Clinical Sciences Lund , Cardiology, Lund University, and the Haemodynamic Laboratory, the Section for Heart Failure and Valvular Disease, VO Heart and Lung Medicine, Skåne University Hospital , Lund , Sweden ;
authors.forename: Göran
authors.initials: G
authors.lastname: Rådegran
authors.affiliation: b Cardiology Unit, Department of Medicine , Karolinska Institute , Stockholm , Sweden ;
authors.forename: Barbro
authors.initials: B
authors.lastname: Kjellström
authors.affiliation: c Department of Public Health and Caring Science , Uppsala University and Uppsala University Hospital , Uppsala , Sweden ;
authors.forename: Björn
authors.initials: B
authors.lastname: Ekmehag
authors.affiliation: d Department of Molecular Medicine and Surgery, Section of Clinical Physiology, Karolinska Institute and Department of Clinical Physiology , Karolinska University Hospital , Stockholm , Sweden ;
authors.forename: Flemming
authors.initials: F
authors.lastname: Larsen
authors.affiliation: e Department of Cardiology , Sahlgrenska University Hospital, the Sahlgrenska Academy, Gothenburg University , Gothenburg , Sweden ;
authors.forename: Bengt
authors.initials: B
authors.lastname: Rundqvist
authors.affiliation: f Department of Medical Sciences Cardiology , Uppsala University , Uppsala , Sweden ;
authors.forename: Sofia Berg
authors.initials: SB
authors.lastname: Blomquist
authors.affiliation: e Department of Cardiology , Sahlgrenska University Hospital, the Sahlgrenska Academy, Gothenburg University , Gothenburg , Sweden ;
authors.forename: Carola
authors.initials: C
authors.lastname: Gustafsson
authors.affiliation: g Department of Clinical Sciences Lund , Rheumatology, Lund University, and the Rheumatology Clinic, Skåne University Hospital , Lund , Sweden ;
authors.forename: Roger
authors.initials: R
authors.lastname: Hesselstrand
authors.affiliation: h Departments of Cardiology and Clinical Physiology , Institution of Medicine and Health Sciences, Linköping University , Linköping , Sweden ;
authors.forename: Monica
authors.initials: M
authors.lastname: Karlsson
authors.affiliation: a Department of Clinical Sciences Lund , Cardiology, Lund University, and the Haemodynamic Laboratory, the Section for Heart Failure and Valvular Disease, VO Heart and Lung Medicine, Skåne University Hospital , Lund , Sweden ;
authors.forename: Björn
authors.initials: B
authors.lastname: Kornhall
authors.affiliation: i Department of Medicine , Karolinska Institute, and the Clinic for Pulmonary Medicine, Karolinska University Hospital , Stockholm , Sweden ;
authors.forename: Magnus
authors.initials: M
authors.lastname: Nisell
authors.affiliation: a Department of Clinical Sciences Lund , Cardiology, Lund University, and the Haemodynamic Laboratory, the Section for Heart Failure and Valvular Disease, VO Heart and Lung Medicine, Skåne University Hospital , Lund , Sweden ;
authors.forename: Liselotte
authors.initials: L
authors.lastname: Persson
authors.affiliation: i Department of Medicine , Karolinska Institute, and the Clinic for Pulmonary Medicine, Karolinska University Hospital , Stockholm , Sweden ;
authors.forename: Henrik
authors.initials: H
authors.lastname: Ryftenius
authors.affiliation: j Department of Public Health and Clinical Medicine , Cardiology and the Heart Centre, Umeå University , Umeå , Sweden ;
authors.forename: Maria
authors.initials: M
authors.lastname: Selin
authors.affiliation: k Department of Cardiology, Karolinska Institute, and Department of Cardiology , Södersjukhuset , Sweden , Stockholm ;
authors.forename: Bengt
authors.initials: B
authors.lastname: Ullman
authors.affiliation: l Department of Clinical Physiology , Örebro University, and Örebro University Hospital , Örebro , Sweden.
authors.forename: Kent
authors.initials: K
authors.lastname: Wall
authors.affiliation: f Department of Medical Sciences Cardiology , Uppsala University , Uppsala , Sweden ;
authors.forename: Gerhard
authors.initials: G
authors.lastname: Wikström
authors.affiliation: f Department of Medical Sciences Cardiology , Uppsala University , Uppsala , Sweden ;
authors.forename: Maria
authors.initials: M
authors.lastname: Willehadson
authors.affiliation: h Departments of Cardiology and Clinical Physiology , Institution of Medicine and Health Sciences, Linköping University , Linköping , Sweden ;
authors.forename: Kjell
authors.initials: K
authors.lastname: Jansson
mesh_terms: D000368:Aged; D015897:Comorbidity; D065627:Familial Primary Pulmonary Hypertension / Q000175:diagnosis / Q000453:epidemiology; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D015994:Incidence; D008297:Male; D062072:Patient Acuity; D011655:Pulmonary Embolism / Q000150:complications*; D016019:Survival Analysis; D013548:Sweden / Q000453:epidemiology
keywords: Incident; prevalent; pulmonary hypertension; survival