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Mitral annular disjunction with atrial septal defect in children: An intriguing association
Mridul Agarwal1, Jay Relan1, Neeraj Aggarwal1
1Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, New Delhi, India.
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.
Insights
Mitral annular disjunction (MAD), a rare condition in children, involves separation of the mitral annulus from the heart muscle. This report details two pediatric cases of MAD associated with atrial septal defects (ASD), highlighting management complexities.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiac Imaging
Background:
- Mitral annular disjunction (MAD) is characterized by separation of the mitral annulus from the left ventricular myocardium.
- MAD is frequently associated with mitral valve prolapse (MVP) and linked to ventricular arrhythmias in adults.
- Reports of MAD in pediatric populations are rare.
Observation:
- This report presents two pediatric cases of MAD.
- Both cases exhibited an association with a large atrial septal defect (ASD).
Findings:
- The co-occurrence of MAD and large ASD in children is described.
- This association presents unique diagnostic and therapeutic challenges.
- This is the first documented report of MAD associated with ASD.
Implications:
- The findings underscore the need for further research into MAD in pediatric patients, particularly when co-occurring with ASD.
- Management strategies for MAD, especially in conjunction with ASD, require careful consideration due to a lack of established guidelines.
- This case series may inform future clinical practice and research directions for this rare condition.
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