Abstract
Mitral annular disjunction (MAD) is defined as a separation between the mitral annulus and the left ventricular myocardium and is most often seen in association with mitral valve prolapse (MVP). MAD has been linked to ventricular arrhythmias in adults, independent of MVP. However, it has rarely been reported in children. We, hereby, report two cases of MAD associated with a large atrial septal defect (ASD). Thus far, there are no consensus guidelines for the management of MAD. The additional association of large ASD further complicates the decision-making in these patients. To the best of our knowledge, this is the first report of the association of MAD with ASD. We further discuss the challenges in the management of this condition.