SESSION TITLE: Fellows Cardiovascular Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Apical Hypertrophic Cardiomyopathy (AHCM) is an uncommon morphological variant of HCM in which the hypertrophy of the myocardium predominantly involves the apex of the left ventricle. These patients do not have Left ventricular outflow tract (LVOT) obstruction but may have midventricular obstruction. Most patients with AHCM present with no or mild symptoms, although presentation with angina, HF, myocardial infarction, atrial fibrillation (AF), or ventricular fibrillation has been reported. CASE PRESENTATION: A 49yr old male with PMHx of HTN who presented to ED with VFib arrest, shock x1. Patient was sleeping in bed when his wife noticed him snoring and suddenly stopped breathing, gasped for breath. CPR for ∼10 min. EMS arrived and the patient was found to be Vfib and he was shocked . ROCS was achieved and the patient was communicative enroute, never intubated and was hemodynamically stable. Family history was significant for CAD in mother. One sibling with CABG. and another with "Arrhythmia and thick heart muscle". Initial EKG with giant/ deep T inversions in V3-V6 and early repolarization changes in V1,V2 (Figure1). Patient underwent emergent cardiac cath which showed clean coronaries and normal ejection fraction. Spade sign was observed on ventriculogram which is characteristic of Apical Hypertrophic Cardiomyopathy (AHCM)(Figure2). AICD was placed on hospital day (HD) 2 for secondary prevention of cardiac arrest. Patient was discharged on HD 4. DISCUSSION: Typical features of apical HCM include•Audible and palpable fourth heart sound, reflecting impaired LV relaxation•"Giant” negative T waves on electrocardiogram (ECG), particularly in the left precordial leads•"Spade-like” configuration of the LV cavity at end-diastole on imaging•Associated apical wall motion abnormalities which may include hypokinesis and aneurysm formation CONCLUSIONS: APCM is an uncommon and underappreciated. High index of suspicion and early recognition on EKG,echocardiography, left ventriculography, most accurately with CMR will aid in early diagnosis, appropriate close follow-up and management. The long-term prognosis for some morphologic subgroups of AHCM might be better than for other HCM phenotypes. However, up to one fourth of individuals can develop significant morbidity with heart failure, apical fibrosis, apical aneurysm formation, ventricular tachycardia and cardiac arrest. Periodic lifelong follow-up is recommended for asymptomatic patients with AHCM. Family screening should be considered. Symptomatic patients are initially treated with medical therapy; however, since nearly all patients with apical HCM do not have evidence of LVOT or mid cavity obstruction, septal reduction therapy is almost never an option in those patients. Risk stratification for ventricular tachyarrhythmias and SCD is the same as for other patients with HCM and should be considered for prophylactic AICD placement(Figure 3). Reference #1: Maron BJ. Clinical course and management of hypertrophic cardiomyopathy. N Engl J Med. 2018; 379:655–668. Reference #2: Stainback RF. Apical hypertrophic cardiomyopathy. Tex Heart Inst J. 2012;39(5):747-749. Reference #3: Rowin EJ, Maron MS, Chan RH, et al. Interaction of adverse disease-related pathways in hypertrophic cardiomyopathy. Am J Cardiol 2017;120:2256-2264. DISCLOSURES: no disclosure on file for Byoungchul Kim; No relevant relationships by Ratnam Santoshi, source=Web Response No relevant relationships by Rajendra Shetty, source=Web Response