Antemortem and Post-Mortem Characteristics of Lethal Mitral Valve Prolapse Among All Countywide Sudden Deaths

Identifying Mitral Valve Prolapse at Risk for Ventricular Arrhythmias and Myocardial Fibrosis from 12-lead Electrocardiograms using Deep Learning

“…We have shown both in living individuals and in post-mortem samples, that MVPs with ventricular arrhythmia or SCD do not consistently have replacement fibrosis. 20, 23 In contrast, diffuse interstitial fibrosis is common, with and without significant MR. Diffuse fibrosis (either primary or MR-related), may cause ECG abnormalities even in the absence of LGE, thus reducing the specificity of our CNN. The ECG segments which contributed most strongly to the LGE prediction were related to ventricular activity, possibly reflecting an underlying MVP-related myopathy.…”

“…Given the known association of MVP with myocardial fibrosis, 10, 18, 23 we performed a separate analysis classifying all MVP patients regardless of ComVE status into 2 groups: patients with and without LGE on contrast CMR.…”

“…16, 17 10, 18, 19 However, in imaging studies of living individuals and in post-mortem samples of malignant MVP cases, bileaflet involvement and focal fibrosis are not consistent findings. 10 20, 21 22, 23 Regardless of leaflet involvement or degree of MR, ComVE is detected in 80-100% of MVPs prior to SCA or SCD. ComVE is associated with myocardial fibrosis and is linked to higher all-cause mortality.…”

Prevalence and potential genetic determinants of young sudden unexplained death victims with suspected arrhythmogenic mitral valve prolapse syndrome

“…Of note, these findings are consistent with a recent meta-analysis of 14 post-mortem SCD studies performed over a 25-year period (1991 to 2016) 9 as well as several relatively recent isolated MVP-focused, post-mortem SCD studies that were either not included or published after the meta-analysis by Nalliah et al Collectively, the prevalence of imaging-and/or autopsydetermined isolated MVP across these post-mortem studies has ranged between 3.8% and 11.7%. 6,9,13,14 Furthermore, in the studies by Basso et al 6 , Han et al 13 and Delling et al 14 as well as an isolated MVP pathology series by Garbi et al 15 , which analyzed the histopathological findings present in SCD decedents with isolated MVP, the rate of left ventricular fibrosis J o u r n a l P r e -p r o o f (interstitial, replacement-type, endocardial plaques, etc.) was significantly higher in SCD decedents with isolated MVP (range 79% to 100%) than those without (range 0% to 38%).…”

“…Collectively, the prevalence of imaging- and/or autopsy-determined isolated MVP across these postmortem studies has ranged between 3.8% and 11.7%. 6 , 9 , 11 , 12 Furthermore, in the studies by Basso and colleagues, 6 Han and colleagues, 11 and Delling and colleagues, 12 as well as an isolated MVP pathology series by Garbi and colleagues, 13 which analyzed the histopathological findings present in SCD decedents with isolated MVP, the rate of left ventricular fibrosis (interstitial, replacement-type, endocardial plaques, etc) was significantly higher in SCD decedents with isolated MVP (range 79%–100%) than those without (range 0%–38%). 6 , 11 …”