ApHCM was associated with annual cardiac death rates ranging from 0.5% to 4%, approaching those for classic HCM.
null
ApHCM is a distinct morphological subtype of hypertrophic cardiomyopathy with unique genetic, clinical, and prognostic features that currently lacks specific management guidelines.
Effect estimate: null (95% CI null)
Absolute Event Rate: 0.5% vs 4%
p-value: p=null
ypertrophic cardiomyopathy (HCM) is an umbrella term for a heterogeneous heart muscle disease that was historically (and still is) defined by the detection of left ventricular (LV) hypertrophy (LVH) in the absence of abnormal cardiac loading conditions.Long after this morphological definition was established, the genetic basis of HCM was discovered, and we now know it is predominantly caused by autosomal dominant mutations in sarcomeric protein genes. 1 Several patterns of LVH have been described in HCM: asymmetric septal (here referred to as "classic" HCM), concentric, reverse septal, neutral, and apical (ApHCM), 2 as well as other, rarer LVH variants such as isolated lateral LVH and isolated inferoseptal LVH.Distinguishing between morphological HCM subtypes has conferred little in terms of personalized management strategies, with one distinctive exception: ApHCM.Compared with classic HCM, ApHCM is more sporadic, sarcomere mutations are detected less frequently, there is more atrial fibrillation (AF) and sudden cardiac death (SCD) risk factors differ.No authoritative ApHCM-specific recommendations to guide diagnosis, family screening, and patient risk stratification currently exist.First described in Japan in 1976, 2 ApHCM is exemplified by "giant" negative precordial T-waves on electrocardiography and by "spadelike" configuration of its LV cavity in end diastole. 3This review summarizes the epidemiology, clinical expression, genetics, and prognosis of ApHCM, while also highlighting knowledge gaps. Pathophysiology and Clinical Characteristics EpidemiologyApHCM is not as rare as first thought, accounting for up to 25% of HCM in Asian populations and 1% to 10% in non-Asians. 4Ethnic variation influences prevalence, natural history, and prognosis, and Western sufferers may exhibit a more malignant form.
Hughes et al. (Fri,) conducted a null in apical hypertrophic cardiomyopathy. null vs. null was evaluated on cardiac death rates (null, 95% CI null, p=null). ApHCM was associated with annual cardiac death rates ranging from 0.5% to 4%, approaching those for classic HCM.