Inherited cardiac channelopathies are linked to a heightened risk of sudden cardiac
death. Despite evolving knowledge on different genes for these inherited conditions,
for certain subtypes, such as catecholaminergic polymorphic ventricular tachycardia
syndrome, the specific genetic causes remain unidentified. The research of the pathophysiological
mechanisms underlying catecholaminergic polymorphic ventricular tachycardia syndrome
has been conducted through different in vitro and in vivo models, including genetically
modified animal models, cardiac‐specific transgenic models, pharmacological interventions
in animal models, human‐induced pluripotent stem cell–derived cardiomyocytes in 2‐
and 3‐dimensional cardiac models. Recent research predominantly utilizes human‐induced
pluripotent stem cell–derived cardiomyocytes, focusing on genotype–phenotype correlations
and pharmacological screening. The integration of cutting‐edge techniques such as
clustered regularly interspaced short palindromic repeats/Cas9 genome editing and
3‐dimensional–engineered heart tissues has shed new light on the pathophysiological
mechanisms of catecholaminergic polymorphic ventricular tachycardia, potentially enhancing
drug therapies as part of personalized medicine approaches. This review emphasizes
the diverse insights gained from both in vivo and in vitro studies of catecholaminergic
polymorphic ventricular tachycardia, along with the application of these models in
various research contexts.
