Key result
Mitral annulus disjunction is linked to malignant arrhythmias in heritable aortic disease.
Why the study?
Mitral annulus disjunction has only recently been described in patients with heritable aortic disease and may contribute to ventricular arrhythmias and sudden cardiac death in this population.
Case Report (n=3)
Mitral annulus disjunction may be an under-recognized arrhythmogenic risk marker in patients with heritable aortic disease and mitral valve prolapse.
May prompt arrhythmia surveillance in heritable aortic disease with MAD and MVP; hypothesis-generating and requires prospective validation.
Patients with heritable aortic disease (HAD) have an increased risk of ventricular arrhythmias and sudden cardiac death. Although mitral valve prolapse is common in HAD, mitral annulus disjunction (MAD) has only recently been described in these patients. This under-recognized condition may be a contributing factor to otherwise unexplained ventricular arrhythmias and sudden cardiac death in patients with HAD. This case series describes 3 patients in an adult HAD clinic who have concomitant mitral valve prolapse, MAD, and malignant arrhythmias. These cases may represent a unique disease entity or overlap syndrome, and they introduce MAD as a potential arrhythmogenic risk marker in HAD.
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Comber et al. (2021) conducted a case report in Heritable aortic disease with malignant arrhythmias (n=3). Mitral annulus disjunction and mitral valve prolapse was evaluated on Malignant arrhythmias. Mitral annulus disjunction and mitral valve prolapse were identified in 3 patients with heritable aortic disease and malignant arrhythmias, suggesting MAD as a potential arrhythmogenic risk marker.
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