Open AccessSurvival in an Incident Cohort of Patients with Pulmonary Arterial Hypertension in Denmark
Author(s) -
Korsholm Kasper,
Andersen Asger,
Kirkfeldt Rikke E.,
Hansen Knud N.,
Mellemkjær Søren,
NielsenKudsk Jens Erik
Publication year - 2015
Publication title -
pulmonary circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.791
H-Index - 40
ISSN - 2045-8940
DOI - 10.1086/681270
Subject(s) - medicine , cohort , pulmonary hypertension , prospective cohort study , cohort study , proportional hazards model , danish , confidence interval , survival rate , population , hazard ratio , survival analysis , philosophy , linguistics , environmental health
We aimed to characterize and estimate survival rates in patients diagnosed with pulmonary arterial hypertension (PAH) in western Denmark in the modern management era. All incident cases of PAH were consecutively enrolled in our single‐center prospective cohort study between January 2000 and March 2012. A total of 134 patients fulfilling the inclusion criteria were followed up from first diagnostic right heart catheterization to either death or the end of the study. Kaplan‐Meier survival analysis was used to estimate 1‐, 3‐, and 5‐year survival rates with 95% confidence intervals (CIs). Survival in the total cohort was 86.4% (95% CI, 79.3%–91.2%) after 1 year, 72.9% (95% CI, 64.1%–79.9%) after 3 years, and 65.4% (95% CI, 55.8%–73.4%) after 5 years. Significantly better survival was seen in the group of patients with PAH associated with congenital heart disease than in the group of patients with idiopathic PAH, heritable PAH, connective tissue disease, HIV infection, and portal hypertension. In conclusion, survival rates in the Danish PAH population were similar to or slightly better than survival rates estimated in other modern registries. However, PAH remains a fatal disease, despite modern targeted therapies.