title: Serial surveillance of carcinoid heart disease: factors associated with echocardiographic progression and mortality.
abstract: BACKGROUND
  Carcinoid heart disease is a complication of metastatic neuroendocrine tumours (NETs). We sought to identify factors associated with echocardiographic progression of carcinoid heart disease and death in patients with metastatic NETs.
  
  
  METHODS
  Patients with advanced non-pancreatic NETs and documented liver metastases and/or carcinoid syndrome underwent prospective serial clinical, biochemical, echocardiographic and radiological assessment. Patients were categorised as carcinoid heart disease progressors, non-progressors or deceased. Multinomial regression was used to assess the univariate association between variables and carcinoid heart disease progression.
  
  
  RESULTS
  One hundred and thirty-seven patients were included. Thirteen patients (9%) were progressors, 95 (69%) non-progressors and 29 (21%) patients deceased. Baseline median levels of serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and plasma 5-hydroxyindoleacetic acid (5-HIAA) were significantly higher in the progressors. Every 100 nmol l(-1) increase in 5-HIAA yielded a 5% greater odds of disease progression (OR 1.05, 95% CI: 1.01, 1.09; P=0.012) and a 7% greater odds of death (OR 1.07, 95% CI: 1.03, 1.10; P=0.001). A 100 ng l(-1) increase in NT-proBNP did not increase the risk of progression, but did increase the risk of death by 11%.
  
  
  CONCLUSIONS
  The biochemical burden of disease, in particular baseline plasma 5-HIAA concentration, is independently associated with carcinoid heart disease progression and death. Clinical and radiological factors are less useful prognostic indicators of carcinoid heart disease progression and/or death.
authors.affiliation: 1] Neuroendocrine Tumour Group, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK [2] Department of Obesity & Endocrinology, University of Liverpool, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK.
authors.forename: R
authors.initials: R
authors.lastname: Dobson
authors.affiliation: 1] Neuroendocrine Tumour Group, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK [2] Department of Cardiology, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK.
authors.forename: M I
authors.initials: MI
authors.lastname: Burgess
authors.affiliation: Manchester Academic Health Sciences Centre, Department of Medical Oncology, The Christie NHS Foundation Trust, Wilmslow Road, Withington, Manchester M20 4BX, UK.
authors.forename: J W
authors.initials: JW
authors.lastname: Valle
authors.affiliation: 1] Department of Gastroenterology, Institute of Translational Medicine, University of Liverpool, Liverpool L69 3GE, UK [2] Neuroendocrine Tumour Group, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
authors.forename: D M
authors.initials: DM
authors.lastname: Pritchard
authors.affiliation: Neuroendocrine Tumour Group, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, UK.
authors.forename: J
authors.initials: J
authors.lastname: Vora
authors.affiliation: Neuroendocrine Tumour Group, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK.
authors.forename: C
authors.initials: C
authors.lastname: Wong
authors.affiliation: Neuroendocrine Tumour Group, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK.
authors.forename: C
authors.initials: C
authors.lastname: Chadwick
authors.affiliation: Department of Clinical Chemistry, University Hospital of South Manchester, Southmoor Road, Wythenshawe, Manchester M23 9LT, UK.
authors.forename: B
authors.initials: B
authors.lastname: Keevi
authors.affiliation: Department of Clinical Chemistry, University Hospital of South Manchester, Southmoor Road, Wythenshawe, Manchester M23 9LT, UK.
authors.forename: J
authors.initials: J
authors.lastname: Adaway
authors.affiliation: Medical Oncology, Huddersfield Royal Infirmary, Lindley, Huddersfield, Yorkshire HD3 3EA, UK.
authors.forename: U
authors.initials: U
authors.lastname: Hofmann
authors.affiliation: Neuroendocrine Tumour Group, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK.
authors.forename: G J
authors.initials: GJ
authors.lastname: Poston
authors.affiliation: 1] Neuroendocrine Tumour Group, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK [2] Department of Obesity & Endocrinology, University of Liverpool, University Hospital Aintree, Lower Lane, Liverpool L9 7AL, UK.
authors.forename: D J
authors.initials: DJ
authors.lastname: Cuthbertson
mesh_terms: D000368:Aged; D002275:Carcinoid Heart Disease / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality*; D018450:Disease Progression; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications* / Q000401:mortality / Q000556:secondary; D008297:Male; D060787:Neoplasm Grading; D018358:Neuroendocrine Tumors / Q000150:complications* / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D011446:Prospective Studies; D015996:Survival Rate