Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA)

TY - JOUR

T1 - Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA)

AU - Olsson, Karen M

AU - Delcroix, Marion

AU - Ghofrani, H Ardeschir

AU - Tiede, Henning

AU - Huscher, Doerte

AU - Speich, Rudolf

AU - Grünig, Ekkehard

AU - Staehler, Gerd

AU - Rosenkranz, Stephan

AU - Halank, Michael

AU - Held, Matthias

AU - Lange, Tobias J

AU - Behr, Juergen

AU - Klose, Hans

AU - Claussen, Martin

AU - Ewert, Ralf

AU - Opitz, Christian F

AU - Vizza, C Dario

AU - Scelsi, Laura

AU - Vonk-Noordegraaf, Anton

AU - Kaemmerer, Harald

AU - Gibbs, J Simon R

AU - Coghlan, Gerry

AU - Pepke-Zaba, Joanna

AU - Schulz, Uwe

AU - Gorenflo, Matthias

AU - Pittrow, David

AU - Hoeper, Marius M

PY - 2014/1/7

Y1 - 2014/1/7

N2 - BACKGROUND: For almost 30 years, anticoagulation has been recommended for patients with idiopathic pulmonary arterial hypertension (IPAH). Supporting evidence, however, is limited, and it is unclear whether this recommendation is still justified in the modern management era and whether it should be extended to patients with other forms of pulmonary arterial hypertension (PAH).METHODS AND RESULTS: We analyzed data from Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), an ongoing European pulmonary hypertension registry. Survival rates of patients with IPAH and other forms of PAH were compared by the use of anticoagulation. The sample consisted of 1283 consecutively enrolled patients with newly diagnosed PAH. Anticoagulation was used in 66% of 800 patients with IPAH and in 43% of 483 patients with other forms of PAH. In patients with IPAH, there was a significantly better 3-year survival (P=0.006) in patients on anticoagulation compared with patients who never received anticoagulation, albeit the patients in the anticoagulation group had more severe disease at baseline. The survival difference at 3 years remained statistically significant (P=0.017) in a matched-pair analysis of n=336 IPAH patients. The beneficial effect of anticoagulation on survival of IPAH patients was confirmed by Cox multivariable regression analysis (hazard ratio, 0.79; 95% confidence interval, 0.66-0.94). In contrast, the use of anticoagulants was not associated with a survival benefit in patients with other forms of PAH.CONCLUSIONS: The present data suggest that the use of anticoagulation is associated with a survival benefit in patients with IPAH, supporting current treatment recommendations. The evidence remains inconclusive for other forms of PAH.CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01347216.

AB - BACKGROUND: For almost 30 years, anticoagulation has been recommended for patients with idiopathic pulmonary arterial hypertension (IPAH). Supporting evidence, however, is limited, and it is unclear whether this recommendation is still justified in the modern management era and whether it should be extended to patients with other forms of pulmonary arterial hypertension (PAH).METHODS AND RESULTS: We analyzed data from Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), an ongoing European pulmonary hypertension registry. Survival rates of patients with IPAH and other forms of PAH were compared by the use of anticoagulation. The sample consisted of 1283 consecutively enrolled patients with newly diagnosed PAH. Anticoagulation was used in 66% of 800 patients with IPAH and in 43% of 483 patients with other forms of PAH. In patients with IPAH, there was a significantly better 3-year survival (P=0.006) in patients on anticoagulation compared with patients who never received anticoagulation, albeit the patients in the anticoagulation group had more severe disease at baseline. The survival difference at 3 years remained statistically significant (P=0.017) in a matched-pair analysis of n=336 IPAH patients. The beneficial effect of anticoagulation on survival of IPAH patients was confirmed by Cox multivariable regression analysis (hazard ratio, 0.79; 95% confidence interval, 0.66-0.94). In contrast, the use of anticoagulants was not associated with a survival benefit in patients with other forms of PAH.CONCLUSIONS: The present data suggest that the use of anticoagulation is associated with a survival benefit in patients with IPAH, supporting current treatment recommendations. The evidence remains inconclusive for other forms of PAH.CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT01347216.

KW - Aged

KW - Anticoagulants

KW - Female

KW - Follow-Up Studies

KW - Hemorrhage

KW - Humans

KW - Hypertension, Pulmonary

KW - Incidence

KW - Male

KW - Middle Aged

KW - Multivariate Analysis

KW - Prognosis

KW - Prospective Studies

KW - Registries

KW - Thrombosis

U2 - 10.1161/CIRCULATIONAHA.113.004526

DO - 10.1161/CIRCULATIONAHA.113.004526

M3 - SCORING: Journal article

C2 - 24081973

VL - 129

SP - 57

EP - 65

JO - CIRCULATION

JF - CIRCULATION

SN - 0009-7322

IS - 1

ER -