Functional Class and Targeted Therapy Are Related to the Survival in Patients with Pulmonary Arterial Hypertension

Purpose: Pulmonary arterial hypertension (PAR) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAR. Materials and Methods: Patients who were diagnosed with PAR at a single center were reviewed retrospectively. Forty patients (34.9 +/- 14.5 years, 80% of female) were enrolled. Results: Causes were congenital heart disease in 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAR in 6 (15%). Sixteen patients (40%) were WHO functional class ifi 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 If were significantly longer than patients with DT 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.