302 CHALLENGING DECISION IN INCIDENTAL APICAL HYPERTROPHIC CARDIOMYOPATHY DIAGNOSIS

Abstract

Abstract Introduction Apical hypertrophic cardiomyopathy (ApHCM) compared with normal HCM is less frequent, it's more associated with atrial fibrillation (AF) and it has different sudden cardiac death (SCD) risk factors. Authoritative recommendations and guidelines on diagnosis, family screening and especially patient risk stratification don't exist. Taking decisions on ICD implantation can sometimes be challenging. Patient presentation A 66-year-old male with a history of arterial hypertension was admitted to our cardiology department because during the last night, while he was driving his car, he would have lost consciousness having a car accident. He rapidly felt well even though he doesn't remember what happened a few minutes shortly before and immediately after the event. No witnesses. Due to traumatic chest pain he went to emergency department where an electrocardiogram was performed with evidence of negative T waves and ST-segment depression on V3-V6 leads. Diagnosis and management Physical examination didn't show anything relevant. On heart ultrasound he had normal left ventricle ejection fraction with apical hypertrophy (maximum thickness 18 mm). He underwent coronary angiography which showed normal coronaries. During hospitalization a cardiac magnetic resonance was performed; it described apical hypertrophy with some small areas of intra-myocardium delayed enhancement in apex wall. During hospitalizations no arrhythmic alarms were detected. Discussion Pharmacological and electrical treatment options are based on classic HCM aiming to reduce symptoms, incidence of atrial fibrillation, ventricular arrhythmias, and sudden death. Concerning ApHCM, left ventricular outflow tract obstruction is usually absent and therefore pharmacological therapeutic benefits may be lower than in classic HCM. Regarding sudden death prevention, we evaluate ICD implantation according to ESC 5-y HCM SCD risk score which doesn't include potential risk markers for SCD in ApHCM such as apical aneurysm, midcavity gradient and midventricular obstruction with SCD risk underestimation. By the way it's still the most used score being aware that could lead to ICD underutilization. In our case, according to HCM risk score 1.74%, negative family screening, no arrhythmic events for 15 days hospitalization, no previous transient loss of consciousness (TLOC) and dynamics of the car accident, we decided to follow-up the patients in our cardiomyopathies outpatient clinic explaining the TLOC with a sleep disorder.