Title A Common Chief Compliant leading to a Unique Diagnosis of Apical Hypertrophic Cardiomyopathy
Authors Brandon Bunol, MD. Hanyuan Shi, MD. Bradley Deere, MD.
Introduction Apical hypertrophic cardiomyopathy (HCM) is a variant that leads to mid-ventricular obstruction instead of LVOT. Most patients with apical HCM tend to be asymptomatic, but can present with insidious symptoms such as angina, heart failure, and syncope. With multimodality imaging, we were able to identify the diseased myocardium in this case and implant a single-chamber ICD for primary prevention, Case Presentation A 55 year-old male with HTN and GERD initially presented with chest pain. He described it as waxing and waning for the last few days. Review of systems notable for multiple episodes of syncope monthly when standing. Vitals showed a BP of 144/95 with a pulse rate of 64 bpm. EKG showed new T-wave inversions in V2, V3 with LVH criteria, with two negative troponins (<0.02 ng/ml). A transthoracic echocardiogram (TTE) resulted with normal LVEF with concentric left ventricular hypertrophy with concern for apical HCM. Coronary angiogram showed mild non-obstructive CAD with intramyocardial bridging of mild LAD. Subsequently, a cardiac MRI was done confirming the diagnosis; hypertrophy of the left ventricle was shown with apical predominance with intraventricular septal thickness at 14 mm with apical segmental scarring (Figure). Beta blockade was initiated, and the patient chose to have a single-chamber ICD implanted for primary prevention of sudden cardiac death. Discussion HC M is an autosomal disease of the myocardium caused by gene mutation in the sarcomere protein. Original diagnostic criteria for apical HCM was a specific “spade - like” configuration with a giant negative T wave with accompanying high QRS voltages on ECG. Now with the advancement of cardiac imaging, TTE and cardiac MRI allow for more accurate assessment of the LVH. In this case, cardiac MRI diagnosed apical HCM with wall dimensions and in the delayed gadolinium enhancement (DGE) phase showing estimated 27 g (14% of LV mass) scar in the apex. Although patients with apical HCM variant have lower risk of arrhythmia, a shared decision was made to place an ICD given patient’s significant history of syncope. Conclusion Apical HCM is a rarer variant but important to recognize as a morphological subtype. Cardiac MRI can better define this phenotype better than TTE alone, and uses LGE to provide better insight into scar patterns.
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