347 ARRHYTHMIC MITRAL VALVE PROLAPSE AND MITRAL ANNULUS DISJUNCTION: WHEN PRIMARY PREVENTION IMPLANTATION OF ICD IS POSSIBLE?

Abstract Mitral valve prolapse (MVP) is the most common valvular heart disease. Although MVP generally considered benign, it can be associated with important complications, including sudden cardiac death (SCD). often related to mitral annular disjunction (MAD), a structural abnormality involving distinct separation of left atrium/mitral annulus and myocardium continuum. It an increased risk arrhythmias integral component arrhythmic (AMVP) complex. Here we present case report patient MAD, Barlow's syndrome bileaflet history recurrent non sustained ventricular tachycardia (NSVT) who underwent subcutaneous cardioverter defibrillator (ICD) implant in primary prevention. A 49 year old woman, moderate regurgitation posterior leaflet prolapse, was sent our hospital by her cardiologist for evidence on holter ecg several tracts arrhythmias. Transesophageal echocardiogram revealed presence regurgitation, (mainly redundant leaflet), dilation atrium positive Pickelhaube sign, suggestive MAD. After few days performed magnetic resonance imaging regurgitation. Delayed gadolinium enhanced (DGE) signal found basal wall. During hospitalization, she presented daily episodes high frequency (>180 bpm) NSVT right bundle block (RBBB) morphology premature therefore decided discharge Life Vest five months later, after asking second opinion Italian specialization center, place ICD prevention patient. Arrhythmic estimated cause death. known that patients MAD have but there aren't trials demonstrate benefits implantation. The recent “EHRA expert consensus statement “ affirms reasonable, implant, AMVP, 1 features (VT (hemodynamically tolerated), NSVT, unexplained syncope) 2 or more phenotypic (—T-wave inversion inferior leads, repetitive documented polymorphic PVCs, phenotype, MV leaflet, enlarged ejection fraction≤50%, LGE). For this reason, subcoutanues implant. Therefore, believe further studies, develop guidelines stratification evident MVP, would beneficial reducing incidence potentially fatal events these patients.