Giant Apical Aneurysm without Coronary Artery Disease

A 66-year-old patient with some years’ history of apical hypertrophic cardiomyopathy treated by beta-blockers attended for follow-up echocardiography. This revealed a giant apical aneurysm as shown in Figure 1A. The obstruction is still visible at the aneurysm outflow, as shown by the aliasing in colour Doppler (arrow, Figure 1B). A significant gradient is present in pulsed waved Doppler (Figure 2). Coronary angiography showed no significant coronary lesion (Figure 3). The ventriculogram confirmed the aneurysm and the obstruction at midventricular level (arrow, Figure 4). The patient died from a non-cardiac cause (lung cancer) two years later.

Apical aneurysm in hypertrophic cardiomyopathy is not a rare finding, and many cases have been reported. It is known that the disease is frequently missed by echocardiography with 57% sensitivity [1]. Magnetic resonance is a good alternative means of diagnosing apical hypertrophy [2] and assessing the presence of apical aneurysm (more commonly found than previously thought [3]). This finding is apparently associated with a poor prognosis and a high rate of sudden death, embolic stroke and heart failure [1,4].

The exact mechanism of aneurysm formation is still unclear, but there is evidence to suggest a high chronic intraventricular pressure gradient due to midventricular obstruction triggering infarction [5,6]. Some other mechanisms may be involved, since magnetic resonance shows diffuse late gadolinium enhancement in these cardiac walls [3].

Management consists of anticoagulant to prevent embolism and consideration of an ICD implantation to prevent sudden cardiac death [1,6].