Elsevier

International Journal of Cardiology

Volume 370, 1 January 2023, Pages 287-293
International Journal of Cardiology

Different characteristics of apical aneurysm in hypertrophic cardiomyopathy are related to difference in long-term prognosis

https://doi.org/10.1016/j.ijcard.2022.09.063Get rights and content

Highlights

  • Overall incidence of AAn in HCM was 9.2%, which was most frequently found in patients with apical and diffuse type of HCM.

  • Patients with AAn showed a higher risk of MACCEs compared with patients without AAn, especially for CVA.

  • AAn with thinning and outpouching of the apex or aneurysmal change of the whole apical segment showed the highest risk of MACCEs.

Abstract

Background

Data regarding long-term cardiac and cerebrovascular adverse events in patients with hypertrophic cardiomyopathy (HCM) and apical aneurysm (AAn) are scarce and specific treatment strategies that include the use of anticoagulants have not yet been established. We aimed to evaluate the prevalence and long-term prognostic implication based on characteristics of AAn in patients with HCM.

Methods

A total of 458 consecutive patients diagnosed with HCM underwent cardiovascular magnetic resonance imaging and echocardiography from August 1, 2008 to December 31, 2015. AAn was classified into Grade 1 and Grade 2 based on size and morphology. The patients were followed up for a median duration of 6.3 years (range, 4.2–8.7 years) for major adverse cardiac and cerebral events (MACCEs); a composite of cardiac death, HCM-related hospitalization, cerebrovascular accident (CVA), heart transplantation, myocardial infarction, and implantable cardiac defibrillator/cardiac resynchronization therapy.

Results

AAn was detected in 9.2%. MACCEs developed more frequently in patients with AAn than in those without AAn (30.1% vs. 20.7%, P = 0.015), with the rate of CVA as the main difference (9.7% vs. 5.3%, P = 0.011). Grade 2 AAn group showed significantly higher MACCE than Grade 1 AAn group (41.8% vs. 21.9%, P < 0.001). In multivariate analysis, the presence of AAn was independently associated with increased risk of MACCEs (adjusted hazard ratio: 1.95; 95% confidence interval, CI: 1.16–3.28; P = 0.012).

Conclusions

AAn is independently associated with increased risk of HCM-related adverse events, especially cerebral infarction, with significant relationship between aneurysm size and adverse events.

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.

Section snippets

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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