Ashwin Srivatsav, Tam Dan Pham, Carolyn Altman, Tam T Doan
Mitral annular disjunction (MAD) is associated with ventricular arrhythmias and sudden cardiac arrest (SCA). Echocardiographic characterization of pediatric MAD-associated SCA remains limited. Among 35 patients aged <21 years with MAD (1/2023-12/2024), 4 had SCA (3 with idiopathic ventricular fibrillation and 1 with an implantable cardioverter-defibrillator shock following an episode of polymorphic ventricular tachycardia). All echocardiograms and clinical data were reviewed. Three patients had moderate-to-severe ventricular dysfunction following cardiac arrest. MAD was not identified on the postarrest echocardiogram in the setting of dysfunction but became apparent after functional recovery. One of the 4 patients had an echocardiogram before their arrest, which demonstrated MAD. All 4 patients continued to have MAD at follow-up. This suggests that in pediatric SCA, moderate-to-severe left ventricular dysfunction may mask MAD in the postarrest period. Serial imaging after functional recovery is important to identify MAD in these patients.