Apical aneurysm formation in apical hypertrophic cardiomyopathy: Pilot study with cardiac magnetic resonance

doi:10.1016/j.ijcard.2022.09.024

International Journal of Cardiology

Volume 371, 15 January 2023, Pages 480-485

https://doi.org/10.1016/j.ijcard.2022.09.024 Get rights and content

Highlights

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There was a 4-step pathway of apical aneurysm formation in apical HCM.
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The extent of hypertrophy and LGE increased and LVEF decreased during the progression of apical HCM.
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Apical HCM is also associated with a high rate of adverse cardiovascular events and further studies are still needed.

Abstract

Background

The dynamic changes and apical aneurysm formation in apical hypertrophic cardiomyopathy (HCM) have not been specifically described. This study aimed to describe these changes to better understand the progression of apical HCM.

Methods and results

Seventy-two patients with apical HCM who underwent at least two cardiac magnetic resonance (CMR) examinations were retrospectively included in this study. The mean interval between the first and last CMR examinations was 50.1 ± 26.8 months (ranging from 4 to 118 months). Compared with the initial values, the left atrial diameter, maximum left ventricular wall thickness and late gadolinium enhancement extent significantly increased (all P < 0.05), while the left ventricular ejection fraction significantly decreased (P < 0.05), at the latest CMR examination. More importantly, the dynamic process of apical aneurysm formation in apical HCM was observed in a subset of patients, which may follow these four stages: starting with systolic apical cavity obliteration, then broadening of the apical slit in systole, further developing into an apical outpouching, and finally forming an apical aneurysm. Eleven patients experienced adverse cardiovascular events, including new-onset or progressive atrial fibrillation (n = 7), hospitalization with heart failure (n = 3) and implantable cardioverter defibrillator intervention (n = 1), at the time of the latest CMR examination.

Conclusions

In the progression of apical HCM, cardiac structure and function will change accordingly. Apical aneurysm formation in apical HCM is a chronic and continuous dynamic process that may follow a 4-step pathway of disease progression.

Introduction

Apical hypertrophic cardiomyopathy (HCM) is a relatively uncommon variant of HCM, accounting for approximately 25% of HCM in Asian populations and 1–10% in non-Asians [1]. It is characterized mainly by apical hypertrophy, a ‘spade-like’ configuration of the left ventricular cavity at end-diastole on left ventriculography, and the presence of precordial T-wave inversion on the electrocardiogram [2]. In the course of apical HCM, the morphology, structure and function of the heart will change accordingly. However, this dynamic process of change and the extent of change have not been specifically described in the literature.

Left ventricular apical aneurysm is a special phenotype in apical HCM, occurring in 2.3% of all apical HCM, as suggested by our previous study [3]. Moreover, our previous study indicated that apical aneurysm in apical HCM is associated with a higher risk of adverse cardiovascular events, including cardiac death, progressive heart failure and new-onset atrial fibrillation (AF) [3]. The mechanism of apical aneurysm formation in apical HCM remains unclear. A few cases of apical aneurysm formation have previously been reported during the long-term follow-up of patients with apical HCM [4,5]. However, the dynamic process of apical aneurysm formation in apical HCM was not recorded in these patients. Therefore, understanding the dynamic process of apical aneurysm formation and intervening in its early stage may delay or prevent the formation of apical aneurysms in apical HCM, thus reducing the occurrence of adverse cardiovascular events.

Therefore, in this study, we aimed to specifically describe the dynamic changes in cardiac structure and function and, more importantly, the dynamic process of apical aneurysm formation in 72 patients with apical HCM who underwent at least two cardiac magnetic resonance (CMR) examinations to better understand the disease progression of apical HCM.

Section snippets

Study population

From January 2010 to December 2019, a total of 1534 patients were diagnosed with apical HCM by CMR imaging in our hospital, and 83 patients who underwent at least two CMR examinations were retrospectively included in this study. The exclusion criteria applied to these patients were i) loss of images from the initial CMR examination (n = 5); ii) cardiac surgery between the first and last CMR examinations (n = 5); and iii) combined with atrial septal defect (ASD) (n = 1). Therefore, a total of 72

Baseline characteristics of the study population

The mean age of the 72 patients at the initial CMR examination was 50.0 ± 13.1 years (ranging from 22 to 80 years). Of the 72 patients, 15 were younger than 40 years old, 42 were between 40 and 60 years old, and the remaining 15 were older than 60 years old. Fifty-five patients (76.4%, 55/72) were men, and 69 patients (95.8%, 69/72) were in New York Heart Association (NYHA) functional class I or II. Fifteen patients (20.8%, 15/72) had no clinical symptoms and visited our hospital due to

Discussion

Apical HCM is a special variant of HCM in which the hypertrophy predominantly affects the left ventricular apex [2]. As the disease progresses, the morphology, structure and function of the heart will change accordingly, though this dynamic process of change has not been specifically described in previous studies. In this study, we described the dynamic changes in cardiac morphology, structure and function in 72 apical HCM patients who underwent at least two CMR examinations, which we hope will

Conclusions

In the progression of apical HCM, the cardiac structure and function will change accordingly, mainly manifested as an increased degree and extent of hypertrophy, increased LGE extent, enlarged left atrium and decreased LVEF. Apical aneurysm formation in apical HCM is a chronic and continuous dynamic process, starting with systolic apical cavity obliteration, then broadening of the apical slit, further apical outpouching and, finally, the formation of the apical aneurysm.

Funding

This study was supported by the National Key Research and Development Program of China (Nos. 2021YFF0501400 and 2021YFF0501404) and the Key Project of the National Natural Science Foundation of China [No. 81930044].

Disclosures

The authors report no relationships that could be construed as a conflict of interest.

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Prevalence, characteristics, and natural history of apical phenotype in a large cohort of patients with hypertrophic cardiomyopathy
2023, Hellenic Journal of Cardiology
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.
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.
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.
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.
Role of Transapical Incision in the Surgical Management of Hypertrophic Cardiomyopathy
2023, Operative Techniques in Thoracic and Cardiovascular Surgery
Transapical ventriculotomy is an important technique in the surgical management of hypertrophic cardiomyopathy (HCM). It is our preferred method for addressing complex long-segment subaortic obstruction and isolated midventricular HCM, as it provides better exposure of the midcavity than alternate methods such as the transaortic or transmitral approach. The procedure can also be used to improve symptoms of diastolic heart failure caused by reduced left ventricular end-diastolic volume as may occur in patients with apical HCM. In this article, we describe our surgical method for transapical myectomy to relieve midventricular obstruction or enlarge the left ventricular cavity to improve diastolic dysfunction in cases of extensive apical hypertrophy.
High-risk imaging characteristics in left ventricular apex for the life-threatening arrhythmic events in Japanese hypertrophic cardiomyopathy patients
2023, Heart and Vessels
Apical hypertrophic cardiomyopathy: pathophysiology, diagnosis and management
2023, Clinical Research in Cardiology

View full text

Apical aneurysm formation in apical hypertrophic cardiomyopathy: Pilot study with cardiac magnetic resonance

Highlights

Abstract

Background

Methods and results

Conclusions

Introduction

Section snippets

Study population

Baseline characteristics of the study population

Discussion

Conclusions

Funding

Disclosures

Am. J. Cardiol.

J. Cardiol. Cases

J. Am. Coll. Cardiol.

J. Am. Coll. Cardiol.

JACC Cardiovasc. Imaging

J. Am. Coll. Cardiol.

J. Cardiol.

J. Am. Soc. Echocardiogr.

J. Am. Coll. Cardiol.

Int. Heart J.

Apical hypertrophic cardiomyopathy: the variant less known

J. Am. Heart Assoc.