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- W4377014599 abstract "Mitral annular disjunction (MAD), which involves an abnormal atrial insertion of the mitral annulus, has been associated with an increased risk for malignant arrhythmias. n/a n/a A 64-year-old female with no significant past medical history had been experiencing symptomatic palpitations along with substernal chest pain following a recent viral illness. She underwent an extensive cardiac workup, including an exercise treadmill test with frequent episodes of NSVT during exercise (Figure 1B) and a transthoracic echocardiogram showing a low-normal ejection fraction with MVP and mitral regurgitation. Ambulatory 24-hour monitor showed rare atrial and ventricular ectopy. Family history was notable for a mother with MVP and an uncle with possible sudden death in his 50’s. A follow up cardiac MRI showed bileaflet MVP, and MAD (Figure 1A). With the findings on the cardiac MRI, along with persistent episodes of symptomatic NSVT, she underwent an EP study to risk stratify and assess need for a primary prevention defibrillator. There was no sustained ventricular tachycardia via burst pacing and programmed stimulation induced during the study, however the study was notable for frequent pleiomorphic ectopy and short bursts of NSVT on high dose isoproterenol similar to the morphology of the ectopy noted on prior stress testing. The patient had an implantable loop recorder placed at the conclusion of the EP study. Genetic testing was performed using an arrhythmia and cardiomyopathy panel and the patient was found to have a likely pathogenic truncation variant of the A-band of the TTN gene (c.87716del, p.Gly29239Aspfs*32). This variant has been identified previously in patients with dilated cardiomyopathy. Surveiilance of the patient’s ILR revealed two episodes of sustained ventricular tachycardia, each lasting 45 seconds in duration (Figure 1C). With these findings, the patient underwent primary prevention dual chamber implantable defibrillator implantation. Patients with MAD and MVP are complex and difficult to risk stratify. Our patient had sustained VT detected by ILR, despite a negative EP study. An interaction with an underlying genetic predisposition to cardiomyopathy may be important." @default.
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- W4377014599 date "2023-05-01" @default.
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- W4377014599 title "PO-05-149 RISK STRATIFICATION FOR VENTRICULAR ARRHYTHMIAS IN MITRAL ANNULAR DISJUNCTION: A CLINICAL CONUNDRUM" @default.
- W4377014599 doi "https://doi.org/10.1016/j.hrthm.2023.03.1441" @default.
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