“Acing” the Hidden Spade: Review of Diagnosis, Follow-up, Prognosis, and Various Associations of Apical Variant Hypertrophic Cardiomyopathy

Abstract

Apical variant hypertrophic cardiomyopathy (AHCM) is a known entity since its first introduction by Sakamoto and Yamaguchi. However, unlike classical hypertrophic cardiomyopathy (HCM), it is less explored in terms of its associated diagnosis and long-term outcomes. Through this case presentation, we aim to have an in-depth review to help physicians identify and better understand several aspects of AHCM. Given the increased availability and utilization of high precision cardiac imaging modalities, apical septal hypertrophic cardiomyopathy will increasingly be recognized as a distinct, clinically significant variant of classical HCM. Contrast echocardiogram is the most effective and diagnostic study when performed in the right setting with high suspicion on clinical examination findings and typical electrocardiogram (EKG) findings. Cardiac magnetic resonance imaging (MRI) has equal diagnostic yield as a contrast echocardiogram. It is associated with a wide spectrum of presentation ranging from asymptomatic course with incidental findings on imaging to rarely being associated with ventricular arrhythmia. The question of utility of implantable defibrillators in individuals, particularly without any underlying arrhythmias, remains unexplained and needs further evidence-based guidance.