title: Detection of sudden death syndromes in New Zealand.
abstract: OBJECTIVE
  To investigate regional variations in the detection of sudden death syndromes across New Zealand by assessing registrations in the national Cardiac Inherited Diseases Registry New Zealand (CIDRNZ).
  
  
  METHODS
  The CIDRNZ has been a national entity since 2009, with a hub in Auckland and locally funded regional coordinators (Midland, Central) linked with multidisciplinary cardiac genetic teams. Registration is consent-based and voluntary, and involves the collection of clinical/genetic information and permits genetic testing and research. Registry data were extracted from the CIDRNZ in October 2015 and results are expressed as registrations per 100,000 people by district health board area.
  
  
  RESULTS
  The CIDRNZ has 1,940 registrants from 712 families, 46% of whom are definitely or probably affected by cardiac inherited disease. There are clear regional differences in registration frequencies between regions and between the North and South Islands, both for overall registrations (56/100,000 and 14/100,000, respectively; p<0.001) and for long QT syndrome registrations (15/100,000 and 6/100,000, respectively; p<0.001). Regions with local coordinators have the highest number of registrations.
  
  
  CONCLUSIONS
  The detection of sudden death syndromes in New Zealand through a cardiac genetic registry is possible but much work is needed to improve regional variation in the detection/reporting of these conditions across the country.
authors.affiliation: Department of Medicine, University of Auckland, Auckland.
authors.forename: Nikki
authors.initials: N
authors.lastname: Earle
authors.affiliation: Paediatric and Congenital Cardiac Services, Starship Children's Hospital, New Zealand.
authors.forename: Jackie
authors.initials: J
authors.lastname: Crawford
authors.affiliation: Genetic Health Service NZ - South Island Hub, Christchurch Hospital, Christchurch.
authors.forename: Kate
authors.initials: K
authors.lastname: Gibson
authors.affiliation: Diagnostic Genetics, LabPlus, Auckland City Hospital, Auckland.
authors.forename: Donald
authors.initials: D
authors.lastname: Love
authors.affiliation: Genetic Health Service NZ - Northern Hub, Auckland City Hospital, Auckland.
authors.forename: Ian
authors.initials: I
authors.lastname: Hayes
authors.affiliation: Genetic Health Service NZ - Central Hub, Wellington Hospital, Wellington.
authors.forename: Katherine
authors.initials: K
authors.lastname: Neas
authors.affiliation: Department of Cardiology, Waikato Hospital, New Zealand.
authors.forename: Martin
authors.initials: M
authors.lastname: Stiles
authors.affiliation: Department of Cardiology, Waikato Hospital, New Zealand.
authors.forename: Mandy
authors.initials: M
authors.lastname: Graham
authors.affiliation: Department of Cardiology, Wellington Hospital, Wellington.
authors.forename: Tom
authors.initials: T
authors.lastname: Donoghue
authors.affiliation: Cardiologist, Department of Cardiology, Wellington Hospital, Wellington.
authors.forename: Andrew
authors.initials: A
authors.lastname: Aitken
authors.affiliation: Department of Paediatrics, Waikato Hospital, New Zealand.
authors.forename: Jon
authors.initials: J
authors.lastname: Skinner
mesh_terms: D017677:Age Distribution; D002423:Cause of Death; D003644:Death Certificates; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000517:prevention & control; D005260:Female; D006297:Health Services Accessibility / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D008403:Mass Screening / Q000379:methods*; D009520:New Zealand / Q000453:epidemiology; D012042:Registries / Q000706:statistics & numerical data*