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

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

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. Systemic hypertension, diabetes, ischemic heart disease and atrial fibrillation were common comorbidities, particularly in those >65 years. One, three and five year survival was 85, 71 and 59% for PAH-patients. Corresponding numbers for CTEPH-patients with vs. without pulmonary endarterectomy were 96, 89 and 86% vs. 91, 75 and 69%, respectively. In 2014, the incidence of IPAH/HPAH, APAH 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 co-morbidities. PAH-survival in SPAHR was similar to other registers.