18 LISTENER FEBRUARY 29 2020
I
t’s easy to think of heart disease as
predominantly a condition of age,
but for one Auckland family, the
opposite is true. Georgia (19), Ruby
(17) and Teina (12) Maoate have all
inherited the syndrome long QT
type 1 from their mother, Mandy. It
means the electrical signal that produces
a heartbeat takes longer to recharge to
initiate the next beat, and puts them at
risk of sudden death from cardiac arrest.
About one in 2000 New Zealanders has
the condition, which is the most commonidentifiable cause of death in young
people who have no other abnormalities
found at autopsy.
Both Georgia and Ruby have already
suffered sudden arrests, but on both
occasions – at home for Georgia after she
ran up the stairs, and at school for Ruby
when she collapsed during a tug of war
in a PE class – their hearts spontaneously
restarted. All three girls have had surgery
to cut the sympathetic nerve under their
left armpit to reduce the risk of exertion
and the “fight or flight” stress responsetriggering an arrest. Even jumping into the
water for a swim or running can activate
the sympathetic response. Ruby has had a
defibrillator implanted because her arrest
happened after her nerve operation and
she was deemed to be at especially high
risk.
Despite passing on the condition to her
daughters, Mandy, who was diagnosed at
the age of 43 after Georgia’s collapse, has
never had an arrest. Her father, aunt and
uncle, however, all died prematurely – at
39, 21 and 10. Tests show her QT intervalThe rhythm of the heart
Cell abnormalities are the focus of local research into a deadly
heart condition that can affect the young and healthy.
REBEKA
H
ROBINSON1