Abstract
Controversies have been raised regarding the prevalence and potential clinical significance of mitral annular disjunction (MAD). We aim to address the anatomic characteristics of MAD and their association, if any, on survival. We retrospectively reviewed 1373 consecutive dissected hearts (1017 men, mean age at death 44.9 ± 0.4 y) and frequently detected MAD (median disjunctional length: 2.0 mm, range: 1.5 mm~8.5 mm), with the prevalence of 92.1% over the entire mitral annulus and 74.9% within the posterior annulus (pMAD). The presence of pMAD was associated with increased all-cause mortality (45 y vs. 49 y, hazard ratio [HR]: 1.28, 95% confidence interval [CI]: 1.11~1.47, p < 0.001), which persisted in the context of cardiovascular diseases (CVDs; 46 y vs. 51 y, HR: 1.33, 95% CI: 1.14~1.56, p < 0.001) but was insignificant in those without CVDs. Compared to those without pMAD, individuals with pMAD affecting the entire posterior annulus or having a mean standardized length of ≥1.78 showed other clinically significant cardiovascular phenotypes, including the enlargement of aortic annular circumferences and a higher occurrence of thoracic aortic aneurysm/dissection. This largest series of autopsies show that MAD is a common phenotype that may exert additive influence on the survival of individuals. It is necessary to establish a precise classification and stratification of MAD.
Highlights
Mitral annular disjunction (MAD) is characterized by an appreciable separation between the mitral valve-atrial wall junction and the ventricular attachment [1]
MAD was demonstrated to be independently associated with ventricular arrhythmias, indicating an underrecognized clinical entity predisposed to sudden cardiac death [4,5]
At posteromedial commissural (PC) and 65.9% at anterolateral commissural (AC)), while the frequencies of MAD were consistent among different regions of the posterior annulus but appeared higher at P1 (49.4%; Table 1)
Summary
Mitral annular disjunction (MAD) is characterized by an appreciable separation between the mitral valve-atrial wall junction and the ventricular attachment [1]. MAD has been widely recognized as a prevalent feature of mitral valve prolapse, associated with the severity of valvular pathology, and predicted to cause ventricular arrhythmias [2,3]. MAD was demonstrated to be independently associated with ventricular arrhythmias, indicating an underrecognized clinical entity predisposed to sudden cardiac death [4,5]. Several independent studies [6,7,8] coincidentally mentioned that MAD appeared associated with younger age at diagnosis. These results motivate additional attention to this special morphological phenotype, MAD, which may have considerable pathophysiological significance. A variable incidence of MAD, ranging from approximately 8% to 98%, has previously been reported in living patients, perhaps reflecting discrepant criteria for diagnosis, study
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