Abstract

Methods: Total 327 patients were enrolled from 2008 to 2011 in the Korean registry program. They met the criteria of PAH and we investigated the clinical data, the medication prescribed and survival status et al. Results: Their mean age was 40.8 15.8years with female /male patient ratio of 2.7:1. The patients in WHO I/II were 32.4% and WHO III/IV were 67.6%. The peak/mean pulmonary arterial pressure was 86.3/ 56.6 mmHg. Idiopathic PAH (22%), connective tissue disease (24%) and congenital heart disease (46%) were predominant. Small number of patients with familial PAH, portal hypertension, HIV infection, pulmonary capillary hemangiomatosis (PCH) existed. The patients described bosentan were 107, sildenafil 50, iloprost 31, and veraprost 49. After follow-up for mean ( SD) duration of 37.2 18.1 months of idiopathic PAH, the survival rates at 1, 2, and 3 years were 70.0%, 64%, and 55% respectively. The survival rates of patients with non-idiopathic PAH were better than that of idiopathic PAH patients. Conclusions: The baseline characteristics and survival rates of our cohort study are close to those of the National Institutes of Health Registry of other countries, and the survival rates of patients with idiopathic PAH were worse than that of non-idiopathic PAH patients. Therefore, the target related therapy should be more popular for the improvement of survival rate of idiopathic PAH patients.

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