Improvement of von Willebrand factor proteolysis after prostacyclin infusion in severe pulmonary arterial hypertension

The presence of dysfunctional von Willebrand factor (vWF) in pulmonary arterial hypertension (PAH) was suggested to be related to increased proteolysis. In 10 patients with severe PAH, we studied the proteolysis of plasma vWF (vWF levels, multimeric distribution, proteolytic pattern, and cleaving pr...

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Bibliographic Details
Published in:Circulation (New York, N.Y.) N.Y.), 2000-11, Vol.102 (20), p.2460-2462
Main Authors: VEYRADIER, Agnès, NISHIKUBO, Toshiya, HUMBERT, Marc, WOLF, Martine, SITBON, Olivier, SIMONNEAU, Gérald, GIRMA, Jean-Pierre, MEYER, Dominique
Format: Article
Language:English
Subjects:
ADAM Proteins
ADAMTS13 Protein
Adult
Antihypertensive Agents - administration & dosage
Biological and medical sciences
Blood Pressure - drug effects
Drug Administration Schedule
Epoprostenol - administration & dosage
Female
Heart Function Tests - drug effects
Hemodynamics - drug effects
Humans
Hypertension, Pulmonary - drug therapy
Hypertension, Pulmonary - physiopathology
Infusions, Intravenous
Male
Medical sciences
Metalloendopeptidases - blood
Peptide Fragments - blood
Pneumology
Pulmonary Artery - physiopathology
Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
Treatment Outcome
Vascular Resistance - drug effects
von Willebrand Factor - metabolism
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Summary:The presence of dysfunctional von Willebrand factor (vWF) in pulmonary arterial hypertension (PAH) was suggested to be related to increased proteolysis. In 10 patients with severe PAH, we studied the proteolysis of plasma vWF (vWF levels, multimeric distribution, proteolytic pattern, and cleaving protease activity) and hemodynamic variables (mean pulmonary artery pressure, cardiac index, and total pulmonary vascular resistance) at baseline and 30 days after initiation of continuous prostacyclin infusion. At baseline, vWF levels were significantly increased, vWF proteolysis was excessive, and vWF-cleaving protease activity remained normal. These biological abnormalities were reversible and paralleled the improvement of hemodynamics under vasodilator treatment with prostacyclin. The excessive proteolysis of vWF in PAH is likely to be related to an increased susceptibility of vWF to proteases induced by high shear rates rather than to an enhanced release of enzymes.
ISSN:0009-7322
1524-4539
DOI:10.1161/01.cir.102.20.2460