Rare case of mitral annulus disjunction and noncompaction-like myocardium

摘要

A 74-year-old man presented to the cardiology office with exertional dyspnea. Transthoracicechocardiography showed mild left ventricular (LV)dilation, LV hypertrophy, LV ejection fraction 50%,mitral valve prolapse and bicuspid aortic valve.Stress echocardiography was nonrevealing. Coronary angiography showed non-obstructive coronary artery disease. Cardiac magnetic resonanceimaging was performed, and showed a 9.1 mm atrialdisplacement of the posterior mitral valve leafletat the hinge point during systole; the finding wasconsistent with mitral annulus disjunction (Fig. 1A,Suppl. Video 1). Cardiac magnetic resonanceimaging also showed spongy/noncompacted myocardium in the LV, with a noncompaction-to-compaction ratio of 1.86 during end-diastole (Fig. 1B).Late gadolinium enhancement images did not showmyocardial fibrosis or presence of LV thrombus.24-hour Holter monitoring showed occasionalpremature ventricular complex, but no ventriculartachycardia.