Apical hypertrophic cardiomyopathy with a plexus of coronary
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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. Serial cardiac biomarkers were negative. Transthoracic echocardiography was performed, and it showed left ventricular apical hypertrophy with preserved systolic function.

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. Further evaluation with pulse-wave Doppler 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. Her hypertrophic cardiomyopathy (HCM) risk score was calculated at 1.8%. She was started on beta-blockers with improvement in her symptoms and no clinical events at 1 year of follow-up.

Coronary artery fistulas (CAFs) are reported to occur in about 0.2% of patients undergoing cardiac catheterization and most of those drain into the right ventricle. Those draining into the left ventricle are very rare and the presence of many of them in the same patient is extremely uncommon. In general, CAFs are thought to represent the persistence of the embryonic intertrabecular vascular network manifesting as prominent Thebesian vessels. They usually present with angina that is thought to be secondary to coronary steal and ischaemia. There have been few reported cases of CAF being associate d with HCM. There are also rare cases of CAF being associated with non-compaction cardiomyopathy.

Source: https://academic.oup.com/ehjcr/article/5/7/ytab249/6323501
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