Skip to main content
. 2018 Aug 9;3(15):e121153. doi: 10.1172/jci.insight.121153

Figure 10. Progressive cardiovascular consequences of Cantu syndrome.

Figure 10

(A) The primary consequence of KATP GOF in the vasculature is reduced excitability, leading to functional and structural vasodilation, low blood pressure, and underperfusion. Secondarily, this leads to a compensatory cardiac hypertrophy and hypercontractility. (B) These features are present in even mild (e.g., SUR2wt/AV) CS models, but are exacerbated in more severe genotypes (e.g., Kir6.1wt/VM and SUR2AV/AV). The latter genotypes are associated with premature death, and very early death occurs immediately after weaning in the most severe genotypes (e.g., SUR2wt/AV/Kir6.1wt/VM and Kir6.1VM/VM).