Apical hypertrophic cardiomyopathy, are low-risk patients really at low risk? A case report

Abstract

Background Hypertrophic cardiomyopathy (HCM) is a genetically determined myocardial disease that constitutes the main cause of sudden cardiac death (SCD) in young athletes. Apical HCM (ApHCM) represents a complex subset of patients, whose risk of SCD seems not negligible. Most applied scores likely underestimate the risk of heart events in this subset of patients. Case summary We report the case of a 55-year-old man who was admitted in the emergency department after an episode of aborted sudden death due to ventricular fibrillation. The electrocardiogram made at admission was noted for atrial fibrillation and a new-onset left bundle branch block. Emergency coronary angiography was normal. The electrocardiogram was repeated and showed symmetrical and profound inversion of T waves in the lateral leads. Transthoracic echocardiogram and cardiac magnetic resonance revealed left ventricular apical hypertrophy suggestive of apical variant of HCM. A cardiac defibrillator was implanted for secondary prevention of SCD. After 6 months of follow-up no further rhythmic events were noted. Discussion Although low, the risk of SCD of ApHCM patients is not negligible. This case illustrates the need for searching of new predictors of rhythmic risk in patients with ApHCM.

The Ace of Spades: Apical Hypertrophic Cardiomyopathy in an African Male Patient

“…Apical hypertrophic cardiomyopathy (Ap HCM) is a rare variant of HCM, and was first described in a case report published in Japan in 1976 [ 1 , 3 , 4 , 5 ]. In the largest study of a cohort of patients with HCM who had undergone investigation, a <10% prevalence of Ap HCM was noted [ 1 , 6 , 7 , 8 ]. The mean age of presentation of Ap HCM is 41 ± 14.5 years, and has a male to female ratio of 1:5 [ 9 ].…”