Inheritance of Imaging Parameters of Arrhythmic Risk in Mitral Valve Prolapse: A Pedigree Study | Circulation: Cardiovascular Imaging
BACKGROUND: A subset of patients with mitral valve prolapse (MVP), a highly heritable condition, experience sudden cardiac arrest (SCA) or sudden cardiac death (SCD). However, the inheritance of phenotypic imaging features of arrhythmic MVP remains unknown. METHODS: We recruited 23 MVP probands, including 9 with SCA/SCD and 14 with frequent/complex ventricular ectopy. Participants underwent interviews, 2-dimensional and speckle-tracking echocardiography, and 48-hour Holter/event monitoring. Each individual was categorized as having a normal mitral valve, MVP, or borderline MVP. We assessed mitral annular disjunction, curling, global longitudinal strain, segmental peak longitudinal strain, electrical/mechanical dispersion, and the postsystolic shortening index in family members and unrelated healthy controls. RESULTS: We enrolled 23 pedigrees (14 extended pedigrees, 4 trios, and 5 duos) with a total of 121 participants (mean age 45 years, 50% women). Multigenerational SCA/SCD occurred i
