title: Pulmonary arterial hypertension in patients with slow-flow vascular malformations.
abstract: 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).
  
  
  METHODS
  Case-control.
  
  
  METHODS
  Multidisciplinary center for vascular anomalies.
  
  
  METHODS
  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.
  
  
  METHODS
  Standard 2-dimensional transthoracic Doppler echocardiography. Venous samples were obtained the same day that echocardiography was performed.
  
  
  METHODS
  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.affiliation: Department of Cardiology, University Clinic of Navarra, Pamplona, Spain.
authors.forename: Moisés
authors.initials: M
authors.lastname: Rodríguez-Mañero
authors.forename: Leyre
authors.initials: L
authors.lastname: Aguado
authors.forename: Pedro
authors.initials: P
authors.lastname: Redondo
mesh_terms: D000293:Adolescent; D000328:Adult; D001783:Blood Flow Velocity / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000378:metabolism; D005340:Fibrinogen / Q000378:metabolism; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D042601:Lymphatic Vessels / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011669:Pulmonary Wedge Pressure / Q000502:physiology*; D012189:Retrospective Studies; D018608:Ultrasonography, Doppler; D054079:Vascular Malformations / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging; D014680:Veins / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology*; D055815:Young Adult; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D005338:Fibrin Fibrinogen Degradation Products; C036309:fibrin fragment D; D014841:von Willebrand Factor; D005340:Fibrinogen