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

• Published in: Yonsei medical journal Vol. 55; no. 6; pp. 1526 - 1532
• Main Authors: Park, Yae Min, Chung, Wook-Jin, Choi, Deok Young, Baek, Han Joo, Jung, Sung Hwan, Choi, In Suck, Shin, Eak Kyun
• Format: Journal Article
• Language: English
• Published: Korea (South) Yonsei University College of Medicine 01.11.2014; 연세대학교의과대학
• Subjects: Adult; Antihypertensive Agents - therapeutic use; Disease Management; Familial Primary Pulmonary Hypertension; Female; Heart Defects, Congenital - complications; Humans; Hypertension - complications; Hypertension, Pulmonary - classification; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - mortality; Kaplan-Meier Estimate; Male; Middle Aged; Molecular Targeted Therapy - methods; Original; Prognosis; Retrospective Studies; Survival Rate; 의학일반; Pulmonary arterial hypertension; functional classification; molecular targeted therapy; survival
• ISSN: 0513-5796; 1976-2437; 1976-2437
• DOI: 10.3349/ymj.2014.55.6.1526

Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. 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. Causes 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). 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.