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박예민,정욱진,최덕영,백한주,정성환,최인석,신억균 연세대학교의과대학 2014 Yonsei medical journal Vol.55 No.6
Purpose: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. Materials and Methods: Patients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9±14.5 years, 80% of female) were enrolled. Results: Causes were congenital heart diseasein 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAH in 6 (15%). Sixteen patients (40%) were WHO functional class III or IV at the time of diagnosis. Twenty seven patients (67.5%) received molecular targeted therapy. During follow-up (53.6±45.5 months), 10 patients (25%) died and 1-, 2-, and 8 year survival rates were 91.3%, 78.7%, and 66.8%, respectively. As expected, mediansurvival of patients with functional class I or II were significantly longer than patients with III or IV (p=0.041). Interestingly, patients with molecular targeted therapy showed longer survival than conventional therapy (p=0.021). Conclusion: WHO functional class at the time of diagnosis was the strong predictor of survival, and molecular targeted therapy could significantly improve the survival. Therefore,early screening and intensive management would be crucial to improve the prognosis in the patient with PAH.