Abstract

Abstract Introduction in most cases mitral valve prolapse is a benign condition, however in 2% of cases it has been associated with the risk of sudden cardiac death (SCD). Basso et al have demonstrated papillary muscle’s and left ventricle’s inferolateral wall fibrosis on cardiac magnetic resonance imaging (MRI), in patients who died from SCD with mitral valve prolapse. Clinical case A 59–year–old patient was admitted to the Cardiology Department of Pertini Hospital (Rome, Italy) for episodes of vertigo and not sustained ventricular tachycardia in two morphologies at 24–hour ECG monitoring. The patient had negative history for cardiovascular risk factors, but an echocardiogram had demonstrated a moderate mitral valve regurgitation, secondary to valve prolapse with mitral annular disjunction (MAD) (Fig.1). Considering patient‘s age associated with low pre–test probability of coronary artery disease, the patient underwent coronary CT that showed a coronary artery tree free from injury. Cardiac MRI showed increased volumes in the left ventricle and slightly reduced systolic function and valve prolapse of both mitral leaflets determining moderate regurgitation. In addition, intra–myocardial Late Gadolinium Enhancement (LGE) showed fibrosis of the inferior and inferolateral wall with extension to the proximal portion of the inferior papillary muscle (Fig.2). Considering such findings, an intracavitary electrophysiological study (EPS) was performed for an evaluation of the arhythmic burden, by administrating the patient a double extra stimulus from the outflow tract of the right ventricle; the treatment induced a polymorphic ventricular tachycardia, starting from the inferior papillary muscle and degenerating into ventricular fibrillation (Fig.3). Discussion considering all the above findings, a mitral valve prolapse at high risk of malignant arrhythmias has been diagnosed and a subcutaneous cardiac defibrillator was implanted. Conclusions in mitral valve prolapse associated with complex ventricular arrhythmias, evaluation with cardiac MRI increasingly plays a crucial role in prognostic stratification for defibrillator implantation in primary prevention.

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