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
Article
Dans Anglais
| 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.
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
Sujet Principal:
Facteur de von Willebrand
/
Cardiopathies congénitales
/
Hypertension pulmonaire
Type d'étude:
Etude d'étiologie
/
Étude pronostique
/
Facteurs de risque
Limites du sujet:
Adolescent
/
Adulte
/
Femelle
/
Humains
langue:
Anglais
Texte intégral:
Braz. j. med. biol. res
Thème du journal:
Biologie
/
Médicament
Année:
2011
Type:
Article
Pays d'affiliation:
Brésil
/
États-Unis d'Amérique
Institution/Pays d'affiliation:
Fundação Pró-Sangue Hemocentro de São Paulo/BR
/
Universidade de São Paulo/BR
/
University of Chicago/US
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