Catecholaminergic Polymorphic Ventricular Tachycardia: Advancing From Molecular Insights to Preclinical Models
Journal of the American Heart Association,
Год журнала:
2025,
Номер
unknown
Опубликована: Март 13, 2025
Inherited
cardiac
channelopathies
are
linked
to
a
heightened
risk
of
sudden
death.
Despite
evolving
knowledge
on
different
genes
for
these
inherited
conditions,
certain
subtypes,
such
as
catecholaminergic
polymorphic
ventricular
tachycardia
syndrome,
the
specific
genetic
causes
remain
unidentified.
The
research
pathophysiological
mechanisms
underlying
syndrome
has
been
conducted
through
in
vitro
and
vivo
models,
including
genetically
modified
animal
cardiac‐specific
transgenic
pharmacological
interventions
human‐induced
pluripotent
stem
cell–derived
cardiomyocytes
2‐
3‐dimensional
models.
Recent
predominantly
utilizes
cardiomyocytes,
focusing
genotype–phenotype
correlations
screening.
integration
cutting‐edge
techniques
clustered
regularly
interspaced
short
palindromic
repeats/Cas9
genome
editing
3‐dimensional–engineered
heart
tissues
shed
new
light
tachycardia,
potentially
enhancing
drug
therapies
part
personalized
medicine
approaches.
This
review
emphasizes
diverse
insights
gained
from
both
studies
along
with
application
models
various
contexts.
Язык: Английский
Analysis of Factors Relevant to the Severity of Symptoms in Children and Adolescents with Postural Orthostatic Tachycardia Syndrome
Children,
Год журнала:
2025,
Номер
12(4), С. 474 - 474
Опубликована: Апрель 7, 2025
Objectives:
The
current
study
aims
to
investigate
the
factors
associated
with
severity
of
conditions
for
pediatric
cases
postural
orthostatic
tachycardia
syndrome
(POTS).
Methods:
Patients
hospitalized
and
first
diagnosed
POTS
were
retrospectively
included
reviewed.
symptoms
was
evaluated
by
symptom
scores
(SSs).
Multiple
Spearman
correlation
analyses
multiple
linear
regression
used
determine
independently
SS.
divided
into
mild
(SS
≤
P25)
severe
≥
P75)
groups
test
distinguishing
efficiency
candidate
factors.
each
correlated
factor
in
indicating
condition
children
assessed
receiver
operating
characteristic
(ROC)
curve.
Results:
A
series
296
patients
aged
5-17
years
suffering
from
included.
showed
that
corrected
QT
interval
dispersion
(QTcd)
SS
(p
<
0.05).
QTcd
can
be
suggest
symptoms,
area
under
curve
(AUC)
0.986
(95%
CI
0.976-0.997).
At
a
threshold
=
45
ms,
sensitivity
specificity
were,
respectively,
94.0%
91.8%
indication.
Conclusions:
In
POTS,
positively
their
exhibited
strong
indicative
value.
ms
valid
cut-off
value
severity.
Язык: Английский
Ion channel expression in intrinsic cardiac neurons: new players in cardiac channelopathies?
Biochimica et Biophysica Acta (BBA) - Molecular Cell Research,
Год журнала:
2025,
Номер
1872(6), С. 119983 - 119983
Опубликована: Май 8, 2025
The
autonomic
nervous
system
is
an
important
modulator
of
electrical
disorders
observed
in
cardiac
pathologies
through
changes
the
balance
between
sympathetic
and
parasympathetic
tone.
final
common
pathway
for
neuronal
control
resides
intrinsic
(ICNS),
composed
intracardiac
neurons
(ICN),
which
allows
sympathetic-parasympathetic
efferent
interactions
at
sites.
ICNS
a
complex
that
plays
crucial
role
regulation
physiological
parameters
has
been
shown
to
contribute
diseases,
particular
arrhythmias.
It
therefore
understand
molecular
determinants,
such
as
ion
channels,
excitability
their
potential
modulation
pathological
conditions.
This
review
discusses
several
channels
expressed
by
ICN,
including
potassium
(e.g.,
inward
rectifier,
calcium-dependent,
voltage-activated,
muscarinic-sensitive),
voltage-gated
sodium
(VGSC),
calcium
(VGCC),
hyperpolarization-activated
cyclic
nucleotide-gated
(HCN)
Transient
Receptor
Potential
(TRP)
Channels,
involvement
pathologies.
We
highlight
need
further
research
on
ICN
particularly
under
conditions,
develop
therapies
Язык: Английский