title: Determination of the optimal echocardiographic scoring system to quantify carcinoid heart disease.
abstract: BACKGROUND
  Carcinoid heart disease (CHD) is an important complication of metastatic neuroendocrine disease, requiring regular monitoring to enable intervention prior to right heart failure. We aimed to identify the most appropriate echocardiographic scoring systems for the quantitative assessment of CHD.
  
  
  METHODS
  In this prospective study conducted between April and October 2012 in two European Neuroendocrine Tumor Society (ENETS) Centres of Excellence, patients with neuroendocrine tumours with liver metastases and/or carcinoid syndrome underwent transthoracic echocardiography and blood sampling for serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and plasma 5-hydroxyindoleacetic acid (5-HIAA). Each patient was assessed according to six echocardiographic scoring systems. The individual scoring systems' feasibility, observer variability, sensitivity, specificity and correlation with the concentration biomarkers were determined.
  
  
  RESULTS
  100 patients were included; 21% had echocardiographic evidence of CHD. All scores discriminated highly between those with/without CHD, with no single score performing significantly better than another. The severity, determined using all of the scoring systems, correlated with the concentration of both biomarkers, but the strongest correlations were seen between the Bhattacharyya score and serum NT-proBNP.
  
  
  CONCLUSIONS
  All scoring systems are comparable in terms of sensitivity and specificity for the detection of CHD. There is a variation in the feasibility of the scoring systems due to varying complexity of the score components. All scores correlate with NT-proBNP and plasma 5-HIAA. The Westberg score appears to be the most optimal scoring system for use in screening of CHD whereas the more complex scoring systems are more suited to the patient with established disease who may require surgical intervention.
authors.affiliation: Neuroendocrine Tumour Group, University of Liverpool, Liverpool, UK.
authors.forename: Rebecca
authors.initials: R
authors.lastname: Dobson
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Cuthbertson
authors.forename: Julia
authors.initials: J
authors.lastname: Jones
authors.forename: Juan W
authors.initials: JW
authors.lastname: Valle
authors.forename: Brian
authors.initials: B
authors.lastname: Keevil
authors.forename: Carrie
authors.initials: C
authors.lastname: Chadwick
authors.forename: Graeme P
authors.initials: GP
authors.lastname: Poston
authors.forename: Malcolm I
authors.initials: MI
authors.lastname: Burgess
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000097:blood; D002275:Carcinoid Heart Disease / Q000097:blood / Q000175:diagnosis* / Q000000981:diagnostic imaging*; D004452:Echocardiography / Q000379:methods*; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D006897:Hydroxyindoleacetic Acid / Q000097:blood; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D018358:Neuroendocrine Tumors / Q000150:complications*; D015588:Observer Variation; D010446:Peptide Fragments / Q000097:blood; D011446:Prospective Studies; D012107:Research Design; D012189:Retrospective Studies; D012680:Sensitivity and Specificity
chemical_list: D015415:Biomarkers; D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D006897:Hydroxyindoleacetic Acid