Göran Rådegran1, Barbro Kjellström2, Björn Ekmehag3, Flemming Larsen4, Bengt Rundqvist5, Sofia Berg Blomquist6, Carola Gustafsson5, Roger Hesselstrand7, Monica Karlsson8, Björn Kornhall1, Magnus Nisell9, Liselotte Persson1, Henrik Ryftenius9, Maria Selin10, Bengt Ullman11, Kent Wall12, Gerhard Wikström6, Maria Willehadson6, Kjell Jansson8. 1. 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 ; 2. b Cardiology Unit, Department of Medicine , Karolinska Institute , Stockholm , Sweden ; 3. c Department of Public Health and Caring Science , Uppsala University and Uppsala University Hospital , Uppsala , Sweden ; 4. d Department of Molecular Medicine and Surgery, Section of Clinical Physiology, Karolinska Institute and Department of Clinical Physiology , Karolinska University Hospital , Stockholm , Sweden ; 5. e Department of Cardiology , Sahlgrenska University Hospital, the Sahlgrenska Academy, Gothenburg University , Gothenburg , Sweden ; 6. f Department of Medical Sciences Cardiology , Uppsala University , Uppsala , Sweden ; 7. g Department of Clinical Sciences Lund , Rheumatology, Lund University, and the Rheumatology Clinic, Skåne University Hospital , Lund , Sweden ; 8. h Departments of Cardiology and Clinical Physiology , Institution of Medicine and Health Sciences, Linköping University , Linköping , Sweden ; 9. i Department of Medicine , Karolinska Institute, and the Clinic for Pulmonary Medicine, Karolinska University Hospital , Stockholm , Sweden ; 10. j Department of Public Health and Clinical Medicine , Cardiology and the Heart Centre, Umeå University , Umeå , Sweden ; 11. k Department of Cardiology, Karolinska Institute, and Department of Cardiology , Södersjukhuset , Sweden , Stockholm ; 12. l Department of Clinical Physiology , Örebro University, and Örebro University Hospital , Örebro , Sweden.
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 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.
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 CTEPHpatients. DESIGN: Incident PAH and CTEPHpatients 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 CTEPHpatients 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 CTEPHpatients 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: Marius M Hoeper; Tilmann Kramer; Zixuan Pan; Christina A Eichstaedt; Jens Spiesshoefer; Nicola Benjamin; Karen M Olsson; Katrin Meyer; Carmine Dario Vizza; Anton Vonk-Noordegraaf; Oliver Distler; Christian Opitz; J Simon R Gibbs; Marion Delcroix; H Ardeschir Ghofrani; Doerte Huscher; David Pittrow; Stephan Rosenkranz; Ekkehard Grünig Journal: Eur Respir J Date: 2017-08-03 Impact factor: 16.671
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Authors: Ioan Tilea; Dorina Nastasia Petra; Razvan Constantin Serban; Manuela Rozalia Gabor; Mariana Cornelia Tilinca; Leonard Azamfirei; Andreea Varga Journal: Int J Gen Med Date: 2021-07-12