An Uncommon Case of Ischemic Heart Disease

Abstract

<h3>Introduction</h3> Diminutive coronary artery syndrome (DCAS) is rare anomaly, defined as a small luminal diameter of >1 of the major coronary vessels. <h3>Case Report</h3> A previously healthy 5 month old infant presented with acute onset dyspnea. Laboratory and radiographic analysis suggested heart failure and end-organ hypoperfusion. A transthoracic echocardiogram demonstrated a dilated (LVDd z score + 6), and hyper-trabeculated left ventricle (LV) (fig 1) with depressed systolic function (ejection fraction 20-25%). The aortic valve (AV) was bicuspid with normal sized annulus, and without any gradient. Interrogation of the coronary arteries revealed normal origins with hypoplasia of the left main ostium, a short left main segment (fig 2), and turbulence by color doppler. CT angiography revealed near atresia of the left coronary artery (LCA) ostium and the main segment measured <1 mm. Cardiac catheterization confirmed findings and revealed stasis of contrast in the LCA. Patch augmentation of the LCA was performed with ECMO support, and required conversion to Berlin Heart Left Ventricular Assist Device (LVAD). While on LVAD support, recovery of LV function was noted, allowing LVAD explantation and unroofing of the myocardial bridge along the mid- and proximal left anterior descending artery. The patient remains supported on an oral heart failure regimen. <h3>Summary</h3> To our knowledge, our patient is the first reported infant with DCAS presenting with decompensated heart failure, associated with LV non compaction and bicuspid AV. We suggest that DCAS should be evaluated in infants with unexplained cardiomyopathy, as ventricular dysfunction in our patient was likely driven by coronary artery insufficiency.