Apical hypertrophic cardiomyopathy with a plexus of coronary artery to left ventricular fistulae

摘要

A 64-year-old woman with no prior history of medical problems was admitted to our hospital with recurrent episodes of chest discomfort. Her vital signs and cardiac examination were normal. An electrocardiogram showed normal sinus rhythm with deep T-wave inversions in leads V4–V6, and biphasic T-waves in leads II, III, and AVF (Supplementary material online, Figure S1). Serial cardiac biomarkers were negative. Transthoracic echocardiography was performed, and it showed left ventricular apical hypertrophy with preserved systolic function (Figure 1, Video 1). Colour Doppler imaging showed multiple areas of mosaic blood flow in the apex that are connected to the ventricle and giving a unique striate appearance (Video 2). Further evaluation with pulse-wave Doppler (Supplementary material online, Figure S2) demonstrated that these represent the diastolic flow from the epicardium into the left ventricular apical cavity, suggestive of coronary to left ventricular fistulae. Coronary angiography was subsequently performed and showed a large and tortuous left coronary artery (proximal diameter of 8–9 mm) without significant stenosis, but with a plexus of multiple small fistulous connections draining into the left ventricular cavity (Figure 2, Video 3). Her hypertrophic cardiomyopathy (HCM) risk score was calculated at 1.8%.1 She was started on beta-blockers with improvement in her symptoms and no clinical events at 1 year of follow-up.