Functional Class and Targeted Therapy Are Related to the Survival in Patients with Pulmonary Arterial Hypertension
Yonsei Medical Journal
;
: 1526-1532, 2014.
Artigo
em Inglês
| WPRIM
| ID: wpr-221610
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 ANDMETHODS:
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Prognóstico
/
Taxa de Sobrevida
/
Estudos Retrospectivos
/
Gerenciamento Clínico
/
Estimativa de Kaplan-Meier
/
Terapia de Alvo Molecular
/
Hipertensão Pulmonar Primária Familiar
/
Cardiopatias Congênitas
/
Hipertensão
/
Hipertensão Pulmonar
Tipo de estudo:
Estudo observacional
/
Estudo prognóstico
Limite:
Adulto
/
Feminino
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Yonsei Medical Journal
Ano de publicação:
2014
Tipo de documento:
Artigo
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