Clinical Spectrum and Outcomes in Hypertrophic Cardiomyopathy With Apical Aneurysms: A Large Multicenter International Cohort.

Authors

• Ethan J. Rowin
• Deacon Z J Lee
• Mark V. Sherrid
• Barry J. Maron
• Albree F. Tower-Rader
• Chiara Zocchi
• Hisham Ahamed
• Aparna Hari
• Alfred J. Albano, Corewell Health West
• Liza Chacko

Document Type

Article

Publication Date

6-2026

Publication Title

JACC. Advances

Abstract

BACKGROUND: Apical aneurysms in hypertrophic cardiomyopathy (HCM) have been linked to sudden cardiac death (SCD) and a nidus for thromboembolism. Uncertainty remains regarding the level of risk and significance of aneurysm size.

OBJECTIVES: The objective of the study was to determine the rate of SCD events and prevalence of apical thrombus or embolic events by size (maximum transverse dimension).

METHODS: Apical aneurysms were identified in 510 patients from 10 centers, followed a median of 4.1 years for SCD events (SCD, appropriate implantable cardioverter defibrillator therapy, and resuscitated SCD) or development of apical thrombus/thromboembolism. Relationship between size and SCD events was analyzed using multivariable Cox proportional hazard models.

RESULTS: In 510 HCM patients: 19% had small aneurysms (< 10 mm), 39% medium (10-19 mm), 39% large (20-39 mm), and 3% very large (≥40 mm). SCD event rate was 2.1%/year, with risk increasing with increasing aneurysm size: 0.2%/year in small, 2.3%/year in medium, 3.0%/year in large and 8.2%/year in very large (P < 0.001). On multivariable analysis, greater size was associated with SCD events, independent of other risk markers or European Society of Cardiology-SCD score. Either an embolic event (3.6% of patients) or apical thrombus (10% of patients) occurred in 13% of patients and was independently associated with greater aneurysm size, 3% in small to 25% in very large aneurysms (P < 0.001).

CONCLUSIONS: In a large cohort of HCM patients with apical aneurysms, rates of SCD events were high, with continuous relationship between size and risk. Small aneurysms (< 10 mm) were associated with low risk for SCD events (0.2%/year), whereas aneurysms ≥10 mm with high risk (>2%/year). Although embolic events were uncommon, increasing aneurysm size was associated with the prevalence of apical thrombi.

Volume

5

Issue

7

First Page

102919

Last Page

102919

Recommended Citation

Rowin EJ, Lee DZJ, Sherrid MV, Maron BJ, Tower-Rader AF, Zocchi C, et al. [Albano AJ]. Clinical spectrum and outcomes in hypertrophic cardiomyopathy with apical aneurysms: A large multicenter international cohort. JACC Adv. 2026;5(7):102919. doi: 10.1016/j.jacadv.2026.102919. PMID: 42308658.

DOI

10.1016/j.jacadv.2026.102919

ISSN

2772-963X

PubMed ID

42308658