Different characteristics of apical aneurysm in hypertrophic cardiomyopathy are related to difference in long-term prognosis
Introduction
Hypertrophic cardiomyopathy (HCM) is one of the most common genetic heart diseases characterized by diverse clinical and phenotypic spectra [1]. Although the typical finding of HCM is asymmetrically hypertrophied myocardium, some patients present with various aneurysmal changes in their dystrophic myocardium, especially at the apex of the left ventricle (LV). This apical aneurysm (AAn) has been described as a discrete thin-walled dyskinetic or akinetic segment of the most distal portion of the chamber with a relatively wide communication with the LV cavity. The prevalence of AAn varies, ranging from 1.3%–8.5% [[2], [3], [4]]. HCM with AAn has reportedly been associated with poor outcomes, in particular, ventricular arrhythmia, and thromboembolic events. Due to these previous studies, recent guidelines for prevention of sudden cardiac death in HCM have incorporated AAn as one of the risk factors of sudden cardiac death [5]. However, data regarding long-term cardiac and cerebrovascular adverse events are scarce and specific treatment strategies for HCM patients with AAn including the use of anticoagulants have not yet been established. Furthermore, the difference in clinical outcomes based on morphology and size of aneurysm remains unknown. A recent retrospective study compared 31 apical HCM patients with and without AAn [6]. They demonstrated the apical HCM patients with AAn had a higher risk of adverse cardiac events than those without AAn. However, this study could not provide information on adverse events other than death or hospitalization because the follow-up period was too short, and did not show a difference according to the type of aneurysm. In addition, since it was reported only for apical HCM patients, it could not represent the overall incidence and risk of AAn in HCM.
Therefore, we aimed to investigate the prevalence and long-term prognostic implication of AAn in all types of HCM using cardiac magnetic resonance imaging (CMR). More importantly, potential difference in clinical findings and cardiac and cerebrovascular outcome was evaluated based on the characteristics of the aneurysm.
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Study design and population
The study population was derived from the institutional HCM registry of Samsung Medical Center, Seoul, Korea. A total of 591 consecutively enrolled patients who were diagnosed with HCM and underwent CMR from August 2008 to December 2015 were included. In this registry dataset, only patients who met the established criteria for HCM and underwent comprehensive clinical, echocardiographic, and CMR evaluation were enrolled. The echocardiographic criteria for HCM were the following: (i) absence of
Prevalence of AAn and baseline characteristics
Among the 458 subjects, the majority were male (80.6%), the mean age was 54.2 ± 11.5 years, and LV AAn was detected in 42 (9.2%) patients. AAn was most frequently detected in subjects with the apical type of HCM (18.9%), followed by diffuse type (11.4%), septal/apical type (7.0%), and septal type (3.0%). Table 1 shows the baseline characteristics based on the presence of AAn.
The age was higher in the AAn group (58.5 [52.8–65.0] years vs. 54.0 [46.3–63.0] years; P = 0.037) and there was a higher
Discussion
In this observational registry, the prognostic implication and prevalence of AAn on CMR in patients with HCM were investigated. Overall incidence of AAn was 9.2%, which was most frequently found in patients with apical and diffuse type of HCM. Regardless of the presence of AF or HCM type, patients with AAn showed a significantly higher risk of MACCEs compared with patients without AAn during long-term follow-up, especially for CVA. More distinct aneurysms that showed wide apical wall thinning
Conclusion
Based on CMR, the prevalence of LV AAn in HCM was 9.2%, which is relatively higher than previously reported. AAn is independently associated with increased risk of HCM-related adverse events, especially cerebral infarction, with a significant prognostic difference based on the extent of aneurysm. Larger prospective studies may be necessary to determine the need for more intensive management such as anticoagulation in HCM patients with AAn.
Author contributions
All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship for this article. All authors contributed to the concept, drafting and critical revision of this article, take responsibility for the integrity of the work as a whole, and have given their approval for this version to be published.
Author statement
All authors have read and approved the final version submitted. This manuscript is not under consideration elsewhere, and none of the contents have been previously published. None of the funding source supported this study.
Financial support
None.
Declaration of Competing Interest
The authors have no conflicts of interest relevant to the submitted work.
Acknowledgements
None.
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