title: Pulmonary arterial thrombosis in eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity.
abstract: OBJECTIVE
  This study sought to determine what factors are associated with pulmonary artery thrombi in Eisenmenger patients.
  
  
  BACKGROUND
  Pulmonary artery thrombosis is common in Eisenmenger syndrome, although its underlying pathophysiology is poorly understood.
  
  
  METHODS
  Adult patients with Eisenmenger syndrome underwent computed tomography pulmonary angiography, cardiac magnetic resonance imaging, and echocardiography. Measurement of ventricular function, pulmonary artery size, and pulmonary artery blood flow were obtained. Hypercoagulability screening and platelet function assays were performed.
  
  
  RESULTS
  Of 55 consecutive patients, 11 (20%) had a detectable thrombus. These patients were older (p = 0.032), but did not differ in oxygen saturation, hemoglobin, or hematocrit from those without thrombus. Right ventricular ejection fraction by magnetic resonance imaging was lower in those with thrombus (0.41 +/- 0.15 vs. 0.53 +/- 0.13, p = 0.017), as was left ventricular ejection fraction (0.48 +/- 0.12 vs. 0.60 +/- 0.09, p = 0.002), a finding corroborated by tissue Doppler and increased brain natriuretic peptide. Those with thrombus also had a larger main pulmonary artery diameter (48 +/- 14 mm vs. 38 +/- 9 mm, p = 0.007) and a lower peak systolic velocity in the pulmonary artery (p = 0.003). There were no differences in clotting factors, platelet function, or bronchial arteries between groups. Logistic regression showed pulmonary artery velocity to be independently associated with thrombosis.
  
  
  CONCLUSIONS
  Pulmonary arterial thrombosis among adults with Eisenmenger syndrome is common and relates to older age, biventricular dysfunction, and slow pulmonary artery blood flow rather than degree of cyanosis or coagulation abnormalities. Further work to define treatment efficacy is needed.
authors: Adult Congenital Heart Disease Centre, Royal Brompton Hospital and National Heart Lung Institute, Imperial College School of Medicine, London, England. brobergc@ohsu.edu
authors: Craig S
authors: CS
authors: Broberg
authors: Masuo
authors: M
authors: Ujita
authors: Sanjay
authors: S
authors: Prasad
authors: Wei
authors: W
authors: Li
authors: Michael
authors: M
authors: Rubens
authors: Bridget E
authors: BE
authors: Bax
authors: Simon J
authors: SJ
authors: Davidson
authors: Beatriz
authors: B
authors: Bouzas
authors: J Simon R
authors: JS
authors: Gibbs
authors: John
authors: J
authors: Burman
authors: Michael A
authors: MA
authors: Gatzoulis
mesh_terms: D000328:Adult; D000367:Age Factors; D001783:Blood Flow Velocity / Q000502:physiology; D003430:Cross-Sectional Studies; D004541:Eisenmenger Complex / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology*; D005260:Female; D006469:Hemoptysis / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011651:Pulmonary Artery; D012307:Risk Factors; D013927:Thrombosis / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D016276:Ventricular Function / Q000502:physiology