• Yae Min Park, Wook-Jin Chung, Deok Young Choi, Han Joo Baek, Sung Hwan Jung, In Suck Choi, and Eak Kyun Shin.
• Division of Cardiology, Heart Center, Gachon University Gil Hospital, Incheon, Korea.; Gachon Cardiovascular Research Institute, Gachon University Gil Hospital, Incheon, Korea.
• Yonsei Med. J. 2014 Nov 1; 55 (6): 1526-32.

PurposePulmonary 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 MethodsPatients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9±14.5 years, 80% of female) were enrolled.ResultsCauses were congenital heart disease in 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, median survival 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).ConclusionWHO 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.

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