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Pulmonary arterial hypertension in patients with slow-flow vascular malformations.
MedLine Citation:
PMID:  21173318     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
OBJECTIVE: To determine the prevalence of pulmonary arterial hypertension in asymptomatic patients with 2 types of extensive slow-flow vascular malformations: extensive venous malformations or Klippel-Trénaunay syndrome (KTS).
DESIGN: Case-control.
SETTING: Multidisciplinary center for vascular anomalies. Patients  A consecutive sample of 32 patients with slow-flow vascular malformations of at least 15% of the body surface was identified retrospectively and matched by age and sex with 32 healthy controls. Interventions  Standard 2-dimensional transthoracic Doppler echocardiography. Venous samples were obtained the same day that echocardiography was performed.
MAIN OUTCOME MEASURES: Pulmonary artery systolic pressure (PASP) was determined. Levels of D-dimer, fibrinogen, and von Willebrand factor (vWF) in plasma were measured.
RESULTS: Patients had a mean (SD) PASP that was significantly higher than that of healthy controls (42.16 [8.49] mm Hg in patients vs 27.69 [6.54] mm Hg in healthy controls; P < .001). No significant differences in PASP were found between patients with KTS and patients with venous malformations (P = .80). We observed significant differences in the mean (SD) levels of vWF between patients and healthy controls (124.41% [52.28%] in patients vs 92.69% [28.92%] in controls; P = .01) and also in levels of D-dimer (1032.99 [1367.0] ng/mL in patients vs 102.97 [29.39] ng/mL in healthy controls; P < .001). There was a moderate positive correlation between levels of vWF and levels of PASP (r = 0.42; P = .001) and a high positive correlation between D-dimer and PASP (r = 0.52; P < .001)
CONCLUSIONS: The presence of pulmonary arterial hypertension in patients with extensive slow-flow vascular malformations is not an isolated feature but is relatively frequent. Levels of D-dimer correlate with PASP in these patients.
Authors:
Moisés Rodríguez-Mañero; Leyre Aguado; Pedro Redondo
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Archives of dermatology     Volume:  146     ISSN:  1538-3652     ISO Abbreviation:  Arch Dermatol     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-12-21     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372433     Medline TA:  Arch Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1347-52     Citation Subset:  AIM; IM    
Affiliation:
Department of Dermatology, University Clinic of Navarra, 31008 Pamplona, Spain. predondo@unav.es.
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