BACKGROUND/AIMS: Idiopathic pulmonary arterial hypertension (IPAH) is an incurable disease with high mortality. Although most studies recommend anticoagulation treatment for IPAH, the benefits are uncertain, particularly in Korea, where it has not been studied. The purpose of this study was to evaluate survival outcomes of Korean patients with IPAH treated with warfarin. METHODS: We performed a retrospective cohort study of patients diagnosed previously with pulmonary arterial hypertension (PAH) at the Asan Medical Center in Korea, between January 1994 and February 2013. We excluded patients with associated PAH, patients who did not undergo right heart catheterization (RHC), and patients with a positive vasoreactivity test. Patients in the study cohort were classified into a "warfarin group" and a "non-warfarin group," according to the treatment they received during the first year after diagnosis. RESULTS: We identified 31 patients with IPAH and a negative vasoreactivity test on RHC. Median patient age was 36.0 years, and 23 patients (74.2%) were female. The median time from the onset of symptoms to diagnosis was 19.0 months, and the most common presenting symptom was dyspnea. Survival rates of the patients at 1, 3, 5, and 10 years were 90.2%, 79.5%, 62.7%, and 34.8%, respectively. The mean survival period was 12.0 years in the warfarin group and 6.1 years in the non-warfarin group. Warfarin treatment had significant survival benefits in patients with IPAH (p = 0.023). CONCLUSIONS: Warfarin treatment substantially improved survival outcomes in Korean cases of IPAH.
Adult ; Anticoagulants/*therapeutic use ; Asian Continental Ancestry Group ; Familial Primary Pulmonary Hypertension/diagnosis/*drug therapy/ethnology/mortality ; Female ; Humans ; Kaplan-Meier Estimate ; Male ; Proportional Hazards Models ; Protective Factors ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Time Factors ; Treatment Outcome ; Warfarin/*therapeutic use