Abstract
Hypertrophic cardiomyopathy (HCM) is characterized by increased left ventricular wall thickness in the absence of any other identifiable cause of thickness. It predisposes the patient to increased risk of sudden cardiac death (SCD) due to fatal arrhythmias. Approximately 2% of the HCM patients have left ventricular apical aneurysm. CMR imaging is better in identifying this apical aneurysm as compared to echocardiogram. This apical aneurysm, which can be akinetic or dyskinetic, increases the risk of disease-related adverse events as compared to general HCM. These adverse disease-related events include SCD, thromboembolism, and symptoms of heart failure. We report a rare case of hypertrophic cardiomyopathy in association with Williams-Beuren Syndrome. On CMR imaging, patient was found to have a large apical aneurysm and mid-ventricular obstruction with underlying thrombus. He was started on oral anticoagulation, and ICD was recommended.