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

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.