Prevalence, characteristics, and natural history of apical phenotype in a large cohort of patients with hypertrophic cardiomyopathy

Pavlos Rouskas; Thomas Zegkos; Dimitris Ntelios; Thomas Gossios; Despoina Parcharidou; Christos A Papanastasiou; Theodoros Karamitsos; Vassilis Vassilikos; Kostantinos Kouskouras; Georgios K Efthimiadis

doi:10.1016/j.hjc.2023.02.004

Prevalence, characteristics, and natural history of apical phenotype in a large cohort of patients with hypertrophic cardiomyopathy

Hellenic J Cardiol. 2023 Sep-Oct:73:8-15. doi: 10.1016/j.hjc.2023.02.004. Epub 2023 Feb 11.

Affiliations

¹ First Cardiology Department, AHEPA University Hospital, Thessaloniki, Greece.
² First Cardiology Department, AHEPA University Hospital, Thessaloniki, Greece. Electronic address: zegkosth@gmail.com.
³ Third Cardiology Department, HIPPOKRATION University Hospital, Thessaloniki, Greece.
⁴ Department of Radiology, University General Hospital of Thessaloniki AHEPA, Thessaloniki, Greece.

PMID: 36775018
DOI: 10.1016/j.hjc.2023.02.004

Abstract

Background: Apical hypertrophic cardiomyopathy (ApHCM) is a variant of hypertrophic cardiomyopathy (HCM) with distinct imaging and clinical characteristics. Data on the prognosis of this HCM subgroup appear conflicting. Our study aims to clarify the natural history of ApHCM and identify predictors of outcomes.

Materials and methods: A total of 856 patients with HCM were retrospectively examined. ApHCM was defined as asymmetric left ventricular hypertrophy confined predominantly at the apex, either isolated (pure ApHCM type) or with co-existent hypertrophy of the interventricular septum (mixed ApHCM). Echocardiographic, clinical, and survival data were compared between individuals with ApHCM and non-ApHCM.

Results: A total of 143 (16.7%) patients were diagnosed with ApHCM. Compared with non-ApHCM, subjects with apical HCM were diagnosed at an older age and had better echocardiographic indices and more comorbidities at baseline. Apical aneurysms were more prevalent among the ApHCM phenotype (6.3% vs. 1.7%, p = 0.003). During a mean follow-up of 6 ± 3 years, ApHCM was characterized by lower all-cause, cardiovascular, heart failure-related mortality, and ventricular arrhythmic events compared with non-ApHCM. Multivariate analysis identified atrial fibrillation and HCM risk-sudden cardiac death (SCD) as independent predictors of the composite outcome of overall mortality and hospitalizations for cardiovascular reasons (hazard ratio [HR] 4.3, 95% confidence interval [CI] 1.9-9.5 for atrial fibrillation and HR 1.2, 95% CI 1.02-1.3 for HCM risk-SCD) in ApHCM.

Conclusions: ApHCM exhibited a lower rate of all-cause mortality and arrhythmic events in the middle-aged population of patients with HCM. Atrial fibrillation and HCM risk-sudden cardiac death were independent predictors of a composite of overall mortality and cardiovascular hospitalizations among those with ApHCM.

Keywords: apical; heart failure; hypertrophic cardiomyopathy; mortality; sudden cardiac death.

MeSH terms

Apical Hypertrophic Cardiomyopathy*
Atrial Fibrillation* / complications
Atrial Fibrillation* / epidemiology
Cardiomyopathy, Hypertrophic* / complications
Cardiomyopathy, Hypertrophic* / diagnosis
Cardiomyopathy, Hypertrophic* / epidemiology
Death, Sudden, Cardiac
Humans
Middle Aged
Phenotype
Prevalence
Prognosis
Retrospective Studies
Risk Factors