Aortic stenosis: correlation of prenatal echocardiography to postmortem histology

Abstract Background Foetal aortic stenosis (AS) is a challenging congenital heart disease considering its potential to progress during the course of pregnancy. Especially at midgestation, it remains extremely difficult distinguish cases that end up biventricular from will develop into an hypoplastic left syndrome. Purpose To test hypothesis degree myocardial maturation possible predictor outcome, we present 4 foetal AS with varying severity and uniquely correlate differences in function based on prenatal echocardiography their post-mortem histopathologic maturation. Methods We selected midgestational our tertiary cardiology service between 2018–2020. Speckle tracking recordings cardiac four-chamber view were performed routine quantify wall motion as marker for function. Three decided terminate pregnancy donated specimen. Immunohistochemical labelling (ICH) against key markers (troponin-I, N-cadherin, connexin-43, MLC2A, MLC2V α-SMA) fibrosis (Sirius Red) compared 2 normal specimens. Results Two critical presented decreased global segmental longitudinal strain (GLS SLS) values −2% −0.9%) ventricle (LV), indicating impaired deformation. Post-mortem ICH showed overt endocardial fibro-elastosis (EFE) pathological patterns subendocardial layer which was remarkably spatially correlated EFE. The cardiomyocytes disorganised reduced expression troponin-I disturbed connexin-43. remaining had LV appearance echocardiography, showing mild reduction ventricular GLS SLS −11.8% −14.2%). 1 these EFE milder pattern. Cardiomyocytes less but also 4th case continued outcome. Conclusions This unique series correlates high extent histology. corresponded amount disorganisation cardiomyocyte network. Myocardial seems hold promise Funding Acknowledgement Type funding sources: None.