Abstract
Long-term anticoagulation is recommended in idiopathic pulmonary arterial hypertension (IPAH). In contrast, limited data support anticoagulation in pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-PAH). We assessed the effect of warfarin anticoagulation on survival in IPAH and SSc-PAH patients enrolled in Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL), a longitudinal registry of group I PAH. Patients who initiated warfarin on study (n=187) were matched 1:1 with patients never on warfarin, by enrollment site, etiology, and diagnosis status. Descriptive analyses were conducted to compare warfarin users and nonusers by etiology. Survival analyses with and without risk adjustment were performed from the time of warfarin initiation or a corresponding quarterly update in matched pairs to avoid immortal time bias. Time-varying covariate models were used as sensitivity analyses. Mean warfarin treatment was 1 year; mean international normalized ratios were 1.9 (IPAH) and 2.0 (SSc-PAH). Two-thirds of patients initiating warfarin discontinued treatment before the last study assessment. There was no survival difference with warfarin in IPAH patients (adjusted hazard ratio, 1.37; P=0.21) or in SSc-PAH patients (adjusted hazard ratio, 1.60; P=0.15) in comparison with matched controls. However, SSc-PAH patients receiving warfarin within the previous year (hazard ratio, 1.57; P=0.031) or any time postbaseline (hazard ratio, 1.49; P=0.046) had increased mortality in comparison with warfarin-naïve patients. No significant survival advantage was observed in IPAH patients who started warfarin. In SSc-PAH patients, long-term warfarin was associated with poorer survival than in patients not receiving warfarin, even after adjusting for confounders. URL: http://www.clinicaltrials.gov. Unique identifier: NCT00370214.
Highlights
Long-term anticoagulation is recommended in idiopathic pulmonary arterial hypertension (IPAH)
No significant survival advantage was observed in IPAH patients who started warfarin
Pulmonary arterial hypertension (PAH) is a fatal disease characterized by a vasoconstrictive, proliferative, thrombotic phenotype, leading to increased pulmonary artery pressure and pulmonary vascular resistance, and eventually to right ventricular failure.[1]
Summary
The design and baseline characteristics of patients enrolled in the REVEAL Registry have been described previously.[21] In brief, the REVEAL Registry is a multicenter (55 sites, university-affiliated and community hospitals), observational, US-based study designed to provide information about demographics, disease course, and management of 3515 consecutively enrolled patients with newly or previously diagnosed group I PAH. The study was conducted in accordance with the amended Declaration of Helsinki and the protocol was reviewed by the institutional review board of each participating center with written informed consent obtained from all patients.[21] Patients were followed for at least 5 years from the time of enrollment. Diagnosis was confirmed by right heart catheterization within 3 months before enrollment for newly diagnosed and >3 months before enrollment for previously diagnosed patients.
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