An Atypical Non-Cardiac Presentation of Hypertrophic Cardiomyopathy.

Abstract

Supplementary Video 2) revealed moderate asymmetric septal LV hypertrophy (interventricular septum thickness 14 mm, posterior wall thickness 9 mm), mildly reduced LV ejection fraction (45%), apical akinesia, and an image suggestive of thrombus, explaining the cardioembolic stroke. Cardiovascular magnetic resonance (CMR) confirmed the diagnosis of HCM, with extensive apical fibrosis and akinesia of the apical segments, outlining an apical aneurysm and thrombus (Figure 2). Coronary angiography was performed regarding the possibility of concomitant ischemic heart disease, ruling out obstructive coronary artery disease. Serologic examination for Trypanosoma cruzi was negative. The patient was started on systemic anticoagulation with a vitamin K antagonist (VKA). Considering the diagnosis of HCM with extensive apical fibrosis and an apical aneurism, after patient-shared decision-making, it was decided to place a subcutaneous implantable cardioverter-defibrillator (S-ICD). He was discharged after 18 days. At the 1-year follow-up, control transthoracic echocardiography showed complete resolution of the apical thrombus, and the patient remained on systemic anticoagulation with a VKA. HCM is a relatively common but still underdiagnosed cardiac disease. 1 This case illustrates an extremely rare and life-threatening non-cardiac first presentation of HCM. This unusual HCM phenotype with a thin-walled, scarred LV apical aneurysm is associated with an increased risk of sudden arrhythmic death and thromboembolic stroke. 2,3