Table 2.
MVP as cause of sudden arrhythmic death: comparison between autopsy- and echo-diagnosed MVP cases
| MVP on autopsy or autopsy + TTE N = 7 | MVP on TTE alone (missed on autopsy) N = 6 | |
|---|---|---|
| Age, years (IQR) | 60 (49–66) | 68 (54–80) |
| Male, n (%) | 5 (71) | 5 (83) |
| Non-white race, n (%) | 2 (29) | 1 (17) |
| MVP as primary cause of SAD, n (%) | 5 (71) | 1 (17) |
| MVP as contributory cause of SAD, n (%) | 2 (29) | 5 (83) |
| Medical History | ||
| Diabetes, n (%) | 0 (0) | 0 (0) |
| Chronic kidney disease, n (%) | 0 (0) | 0 (0) |
| Hypertension, n (%) | 2 (29) | 3 (50) |
| Tobacco use, n (%) | 3 (43) | 2 (33) |
| Drug use, n (%) | 1 (14) | 0 (0) |
| Obstructive CAD, n (%) | 0 (0) | 0 (0) |
| Family History | ||
| SCD with unknown MVP status | 0 (0) | 1 (17) |
| Documented MVP | 1 (14) | 0 (0) |
| SCD + documented MVP | 1 (14) | 0 (0) |
| ECG characteristics | ||
| Pre-mortem 12-lead ECG available | 4 (57) | 5 (83) |
| LBBB | 1 | 1 |
| Inverted or biphasic T waves in inferior ECG leads | 3 | 1 |
| Pre-mortem ambulatory ECG monitoring | 0 | 0 |
| Echocardiographic characteristics | ||
| Pre-mortem TTE available, n (%) | 3 (43) | 6 (100) |
| LVEF, % (IQR) | 58 (54–69) | 64 (50–78) |
| MVP leaflet involvement | ||
| Bileaflet | 1 | 0 |
| Posterior | 2 | 3 |
| Anterior | 0 | 2 |
| Prior mitral valve repair | 0 | 1* |
| ≥ Moderate mitral regurgitation | 2 | 0 |
| Mitral annular disjunction | 2 | 1 |
| Length, mm | 0–8 | 0–7 |
| Tricuspid valve prolapse | 0 | 2 |
| Right ventricular dilatation | 0 | 0 |
| Right ventricular systolic dysfunction | 0 | 0 |
| Gross autopsy findings | ||
| Cardiomegaly, n (%) | 3 (43) | 1 (17) |
| Thick mitral valve leaflets, n (%) | 7 (100) | 6 (100) |
| Mild | 0 (0) | 2 (33) |
| Moderate to severe | 7 (100) | 4 (67) |
| Histologic autopsy findings | ||
| Replacement fibrosis, n (%) | 3 (43) | 1 (17) |
| Inferior-basal LV | 1 | 0 |
| SLPM and subjacent LV wall | 0 | 0 |
| IMPM and subjacent LV wall | 2 | 1 |
| Endoperimysial fibrosis, n (%) | 7 (100) | 6 (100) |
| Inferior-basal LV (mild/mod/severe) | 1 (1/0/0) | 0 (0/0/0) |
| SLPM and subjacent LV (mild/mod/severe) | 2 (1/1/0) | 4 (1/3/0) |
| IMPM and subjacent LV (mild/mod/severe) | 3 (0/0/3) | 4 (0./2/2) |
| Septum (mild/mod/severe) | 2 (1/1/0) | 3 (2/1/0) |
MVP indicates mitral valve prolapse; TTE, transthoracic echocardiogram; SAD, sudden arrhythmic death; CAD, coronary artery disease; SCD, WHO-defined (presumed) sudden cardiac death; ECG, electrocardiogram; LBBB, left bundle branch block; LVEF, left ventricular ejection fraction; LVMI, left ventricular mass index; SLPM and IMPM, superior-lateral and inferior-medial papillary muscle.
*
posterior MVP prior to mitral valve repair. Data shown as median (interquartile range or IQR) and n (%).