P1502 A clinical case of mitral annulus disjunction syndrome revealed by cardiac MRI

Abstract

Abstract A 39year old female patient with a family history of probable sudden cardiac death/long QT syndrome (sister died aged 36) was referred to our Arrhythmia/inherited cardiac conditions clinic. Patient was fit and well. She described occasional postural presyncope and some chest pain with stress. Not on any medication that could prolong QT. On examination she appeared well with normal heart sounds and no clinical signs of heart failure. Her ECG in clinic confirmed sinus rhythm with normal QTc at 418msec. There were U waves which may reflect late repolarisation of His Purkinje system. Diagnostic techniques and findings A 24-hour ECG tape to look at the average QT interval, an exercise tolerance test to look for appropriate shortening of QT interval with exercise and an echocardiogram to exclude significant structural abnormality were requested. The 24-hour ECG tape confirmed underlying sinus rhythm with multi-focal aberrant beats <1% of total. One symptom demonstrated short run of ventricular bigeminy. During the exercise tolerance test, QT interval was not obviously prolonged. There were frequent bifocal ventricular ectopic beats in recovery. The echocardiogram showed minor abnormality with mild left ventricular dilatation, otherwise normal findings. Patient referred for cardiac MRI In order to rule out any cardiomyopathy. Cardiac MRI did not show any prolapse on the 3 chamber cine. In addition, the inferior and inferolateral annulus showed thinning passing into the LV, which is a feature of some patients with mitral annulus disjunction syndrome. We went through the echocardiogram and in one single frame images are suspicious of focal P2 prolapse. Learning points: Mitral annulus disjunction (MAD) is an abnormal atrial displacement of the mitral valve leaflet hinge point. It has been associated with mitral valve prolapse and sudden cardiac death. MAD is usually revealed with echocardiogram. In this case report we showed that CMR revealed a MAD previously missed by echo. Abstract P1502 Figure.