Yonsei Med J.  2014 Nov;55(6):1526-1532. 10.3349/ymj.2014.55.6.1526.

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

• Park YM
• Chung WJ
• Choi DY
• Baek HJ
• Jung SH
• Choi IS
• Shin EK

• KMID: 2070199
• DOI: http://doi.org/10.3349/ymj.2014.55.6.1526

Abstract

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 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).
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.

Keyword

Pulmonary arterial hypertension; survival; functional classification; molecular targeted therapy

MeSH Terms

Adult
Antihypertensive Agents/*therapeutic use
*Disease Management
Familial Primary Pulmonary Hypertension
Female
Heart Defects, Congenital/complications
Humans
Hypertension/complications
Hypertension, Pulmonary/*classification/*drug therapy/mortality
Kaplan-Meier Estimate
Male
Middle Aged
Molecular Targeted Therapy/*methods
Prognosis
Retrospective Studies
Survival Rate
Antihypertensive Agents

Figure

• 

  Fig. 1 Median overall survival time of patients by NYHA functional classification. Patients with NYHA class I or II at the time of diagnosis showed significantly better survival than those with more severe functional class.

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  Fig. 2 Median overall survival time of patients according to the treatment. Patients with molecular targeted therapy showed significantly better survival than those with conventionally therapy only.

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