Plasma von Willebrand factor as a predictor of survival in pulmonary arterial hypertension associated with congenital heart disease
Braz. j. med. biol. res
;
44(12): 1269-1275, Dec. 2011. ilus, tab
Artículo
en Inglés
| LILACS
| ID: lil-606536
ABSTRACT
Biomarkers have been identified for pulmonary arterial hypertension, but are less well defined for specific etiologies such as congenital heart disease-associated pulmonary arterial hypertension (CHDPAH). We measured plasma levels of eight microvascular dysfunction markers in CHDPAH, and tested for associations with survival. A cohort of 46 inoperable CHDPAH patients (age 15.0 to 60.2 years, median 33.5 years, femalemale 2917) was prospectively followed for 0.7 to 4.0 years (median 3.6 years). Plasma levels of von Willebrand factor antigen (VWFAg), tissue plasminogen activator (t-PA) and its inhibitor (PAI-1), P-selectin, reactive C-protein, tumor necrosis factor alpha, and interleukin-6 and -10 were measured at baseline, and at 30, 90, and 180 days in all subjects. Levels of six of the eight proteins were significantly increased in patients versus controls (13 to 106 percent increase, P < 0.003). Interleukin-10 level was 2.06 times normal (P = 0.0003; Th2 cytokine response). Increased levels of four proteins (t-PA, PAI-1, P-selectin, and interleukin-6) correlated with disease severity indices (P < 0.05). Seven patients died during follow-up. An average VWFAg (mean of four determinations) above the level corresponding to the 95th percentile of controls (139 U/dL) was independently associated with a high risk of death (hazard ratio = 6.56, 95 percentCI = 1.46 to 29.4, P = 0.014). Thus, in CHDPAH, microvascular dysfunction appears to involve Th2 inflammatory response. Of the biomarkers studied, plasma vWFAg was independently associated with survival.
Texto completo:
Disponible
Índice:
LILACS (Américas)
Asunto principal:
Factor de von Willebrand
/
Cardiopatías Congénitas
/
Hipertensión Pulmonar
Tipo de estudio:
Estudio de etiología
/
Estudio pronóstico
/
Factores de riesgo
Límite:
Adolescente
/
Adulto
/
Femenino
/
Humanos
Idioma:
Inglés
Revista:
Braz. j. med. biol. res
Asunto de la revista:
Biologia
/
Medicina
Año:
2011
Tipo del documento:
Artículo
País de afiliación:
Brasil
/
Estados Unidos
Institución/País de afiliación:
Fundação Pró-Sangue Hemocentro de São Paulo/BR
/
Universidade de São Paulo/BR
/
University of Chicago/US
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