PO-05-149 RISK STRATIFICATION FOR VENTRICULAR ARRHYTHMIAS IN MITRAL ANNULAR DISJUNCTION: A CLINICAL CONUNDRUM

Mitral annular disjunction (MAD), which involves an abnormal atrial insertion of the mitral annulus, has been associated with increased risk for malignant arrhythmias. n/a A 64-year-old female no significant past medical history had experiencing symptomatic palpitations along substernal chest pain following a recent viral illness. She underwent extensive cardiac workup, including exercise treadmill test frequent episodes NSVT during (Figure 1B) and transthoracic echocardiogram showing low-normal ejection fraction MVP regurgitation. Ambulatory 24-hour monitor showed rare ventricular ectopy. Family was notable mother uncle possible sudden death in his 50’s. follow up MRI bileaflet MVP, MAD 1A). With findings on MRI, persistent NSVT, she EP study to stratify assess need primary prevention defibrillator. There sustained tachycardia via burst pacing programmed stimulation induced study, however pleiomorphic ectopy short bursts high dose isoproterenol similar morphology noted prior stress testing. The patient implantable loop recorder placed at conclusion study. Genetic testing performed using arrhythmia cardiomyopathy panel found have likely pathogenic truncation variant A-band TTN gene (c.87716del, p.Gly29239Aspfs*32). This identified previously patients dilated cardiomyopathy. Surveiilance patient’s ILR revealed two tachycardia, each lasting 45 seconds duration 1C). these findings, dual chamber defibrillator implantation. Patients are complex difficult stratify. Our VT detected by ILR, despite negative An interaction underlying genetic predisposition may be important.