title: The importance of specialist cardiac histopathological examination in the investigation of young sudden cardiac deaths.
abstract: OBJECTIVE
  Post-mortem examination of the heart in young sudden cardiac death (SCD) is vital as the underlying aetiology is often an inherited cardiac disease with implications for surviving relatives. Our aim is to demonstrate the improvement in diagnostic quality offered by a specialist cardiac pathology service established to investigate SCD with fast-track reporting on hearts sent by pathologists in cases of SCD.
  
  
  RESULTS
  A tertiary centre prospective observational study was conducted. Detailed histopathological examination was performed in a tertiary centre specialized in the investigation of cardiac pathology in SCD. Hearts from 720 consecutive cases of SCD referred by coroners and pathologists from 2007 to 2009 were included. A comparison was drawn with diagnoses from referring pathologists. Most SCDs occurred in males (66%), with the median age being 32 years. The majority (57%) of deaths occurred at home. The main diagnoses were a morphologically normal heart (n = 321; 45%), cardiomyopathy (n = 207, 29%), and coronary artery pathology (n = 71; 10%). In 158 out of a sample of 200 consecutive cases, a cardiac examination was also performed by the referring pathologist with a disparity in diagnosis in 41% of the cases (κ = 0.48). Referring pathologists were more inclined to diagnose cardiomyopathy than normality with only 50 out of 80 (63%) normal hearts being described correctly.
  
  
  CONCLUSIONS
  Expert cardiac pathology improves the accuracy of coronial post-mortem diagnoses in young SCD. This is important as the majority of cases may be due to inherited cardiac diseases and the autopsy guides the appropriate cardiological evaluation of blood relatives for their risk of sudden death.
authors.affiliation: CRY Centre for Cardiac Pathology, National Heart and Lung Institute, Imperial College London, SW3 6LY London, UK.
authors.forename: Sofia V
authors.initials: SV
authors.lastname: de Noronha
authors.affiliation: Cardiovascular Sciences Research Centre, St George's University of London, SW17 0RE London, UK.
authors.forename: Elijah R
authors.initials: ER
authors.lastname: Behr
authors.affiliation: Cardiovascular Sciences Research Centre, St George's University of London, SW17 0RE London, UK.
authors.forename: Michael
authors.initials: M
authors.lastname: Papadakis
authors.affiliation: Department of Histopathology, Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, UK.
authors.forename: Keiko
authors.initials: K
authors.lastname: Ohta-Ogo
authors.affiliation: Department of Histopathology, Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, UK.
authors.forename: Winston
authors.initials: W
authors.lastname: Banya
authors.affiliation: CRY Centre for Cardiac Pathology, National Heart and Lung Institute, Imperial College London, SW3 6LY London, UK.
authors.forename: Jemma
authors.initials: J
authors.lastname: Wells
authors.affiliation: CRY, Unit 7, Epsom Downs Metro Centre, Waterfield, Tadworth, Surrey KT20 5LR, UK.
authors.forename: Steve
authors.initials: S
authors.lastname: Cox
authors.affiliation: CRY, Unit 7, Epsom Downs Metro Centre, Waterfield, Tadworth, Surrey KT20 5LR, UK.
authors.forename: Alison
authors.initials: A
authors.lastname: Cox
authors.affiliation: Cardiovascular Sciences Research Centre, St George's University of London, SW17 0RE London, UK.
authors.forename: Sanjay
authors.initials: S
authors.lastname: Sharma
authors.affiliation: CRY Centre for Cardiac Pathology, National Heart and Lung Institute, Imperial College London, SW3 6LY London, UK Department of Histopathology, Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, UK m.sheppard@rbht.nhs.uk.
authors.forename: Mary N
authors.initials: MN
authors.lastname: Sheppard
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D009202:Cardiomyopathies / Q000401:mortality / Q000473:pathology*; D015984:Causality; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D003324:Coronary Artery Disease / Q000401:mortality / Q000473:pathology*; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010336:Pathology; D012017:Referral and Consultation / Q000706:statistics & numerical data*; D017678:Sex Distribution; D006113:United Kingdom; D055815:Young Adult
keywords: Autopsy; Cardiomyopathy; Morphologically normal heart; Sudden arrhythmic death syndrome; Sudden cardiac death; Young