Table 2.
3D Model | Disease/Gene/ Variant |
Cellular Phenotype | Ref. |
---|---|---|---|
Cardiac Microtissue |
ACM PKP2 (c.2013delC, p.(Lys672ArgfsX12)) |
Lower Cx43 expression and arrhythmic behaviour of ACM cMT consisting of control CM and EC and ACM cardiac fibroblasts | [50] |
Cardiac Microtissue |
LQTS KCNQ1 p.(Arg594Gln) |
Prolonged field potential duration (FPD), β-adrenergic stimulation shortened the RR interval and decreased the FPD | [52] |
Engineered heart tissue | LQTS KCNH2 p.(Ala614Val) |
APD prolongation (via ArcLight), re-entrant arrhythmic activity after IKr blocking with dofetilide | [53] |
Engineered heart tissue | CPVT CASQ2 p.(Asp307His) |
More [Ca2+]i transient abnormalities and arrhythmias compared to control EHT but less than single cell CPVT iPSC-CM | [53] |