Long QT molecular autopsy in sudden unexplained death in the young (1-40 years old): Lessons learnt from an eight year experience in New Zealand.
Luciana MarcondesJackie CrawfordNikki EarleWarren SmithIan HayesPaul MorrowTom DonoghueAmanda GrahamDonald LoveJonathan R Skinnernull nullPublished in: PloS one (2018)
In New Zealand, variants in SCN5A and KCNH2, with maternal inheritance, predominate. A rare variant in LQTS genes is more likely in whites rather than Maori, females, children 1-12 years and those with a positive personal and family history of seizures, syncope or SUDY. Family screening supported the diagnosis in a third of the cases. The changing classification of variants creates a significant challenge.