Apical hypertrophic cardiomyopathy with left ventricular apical aneurysm: prevalence, cardiac magnetic resonance characteristics, and prognosis

Abstract Aims Hypertrophic cardiomyopathy (HCM) with left ventricular apical aneurysm (LVAA) is associated with an increased risk of adverse cardiovascular events. However, the clinical significance of LVAA in apical HCM (ApHCM) has not been reported. This study aimed to investigate the prevalence, cardiac magnetic resonance (CMR) characteristics, and prognosis of LVAA in ApHCM patients. Methods and results A total of 1332 consecutive ApHCM patients confirmed by CMR in our hospital were retrospectively analysed. LVAAs were identified in 31 patients of all ApHCM patients (2.3%, 31/1332). Besides, 31 age- and gender-matched ApHCM patients without LVAA were used for comparison. Of the 31 aneurysm patients (mean age, 53.8 ± 15.1 years old), 28 (90.3%) had clinical symptoms, and 3 (9.7%) had a family history of HCM. The rate of missed diagnosis of echocardiography for detecting LVAA was 64.5% (20/31), most (90%, 18/20) of unidentified LVAAs by echocardiography were small aneurysms (<20 mm). Compared with ApHCM patients without LVAA, the proportion of systolic mid-cavity obstruction and late gadolinium enhancement (LGE) presence, and the LGE extent in ApHCM patients with LVAA were significantly higher (all P<0.05). The Kaplan–Meier curves showed that the event-free survival rate in ApHCM patients with LVAA was significantly lower than that in ApHCM patients without LVAA (log rank, P = 0.010). Conclusion ApHCM with LVAA is a very rare condition, which is often missed by echocardiography and could be reliably detected with CMR and is associated with a higher risk of adverse cardiovascular events compared with ApHCM without LVAA.