Calcified Aneurysm of Left Ventricular Apex in Apical Cardiomyopathy

Abstract

WEBSITE FEATURE

A 65-year-old obese, nondiabetic, normotensive woman presented with exertional angina of 4 years' duration. She had experienced a transient ischemic attack 15 days previously. Physical examination yielded nothing unusual. An electrocardiogram showed poor precordial R-wave progression with Q waves in leads V₁ through V₆, II, III, and aVF, and T inversions in the precordial leads (Fig. 1). However, the patient reported no history that suggested acute coronary syndrome.

Fig. 1 Twelve-lead electrocardiogram shows widespread Q waves, T inversions, and attenuated R waves.

An echocardiogram showed normal left ventricular (LV) function, dimensions, and wall thickness; however, the window to evaluate the LV apex was poor. Coronary angiography showed normal coronary arteries (Fig. 2A). Injection of contrast medium into the LV revealed hypercontractile mid-LV myocardium and a spherical, calcified cavity that projected outward and communicated with the LV apex (Fig. 2B).

Fig. 2 A) Coronary angiogram shows normal epicardial coronary arteries with a calcified spherical lesion at the left ventricular apex (arrow). B) Angiogram shows obliteration of the mid-left ventricular cavity (arrowhead) during systole and distortion of the ventricle's classic “ace of spades” appearance by the apical aneurysm (arrow).

Real-time motion image of Figure 2B is available at www.texasheart.org/journal.

Cardiac magnetic resonance (CMR) revealed asymmetric hypertrophy of the LV (interventricular septum, 14 mm; posterior wall, 10 mm). The LV myocardium contracted vigorously except at the apex, which was aneurysmal and calcified (Fig. 3). The apical aneurysm shared myocardium with the surrounding LV. Delayed-enhancement images showed transmural enhancement of the lesion, consistent with fibrosis, and confirmed the presence of mural thrombus (Fig. 4). The final diagnosis of apical hypertrophic cardiomyopathy with apical aneurysm was made on the basis of the CMR findings.

Fig. 3 Single-shot cardiac magnetic resonance images (steady-state free precession) show A) septal hypertrophy and the apical aneurysm (arrow), which shares myocardium with the left ventricle (4-chamber view in diastole); and B) the apical aneurysm (arrow) with persistent mid-cavity obliteration (arrowhead) (vertical long-axis view in systole). The wall of the aneurysm shows calcification (indicated by the very hypointense rim), and the cavity contains thrombus (indicated by the inner iso- to hypointense contents).

Real-time motion image of Figure 3B is available at www.texasheart.org/journal.

Fig. 4 Delayed-enhancement cardiac magnetic resonance shows enhanced margins of the lesion in A) vertical long-axis and B) 4-chamber views, suggesting fibrosis and hypointense intracavitary thrombus (arrow).

Comment

Apical aneurysm in the absence of epicardial coronary artery disease is a rare sequela of apical hypertrophic cardiomyopathy.¹ High intraventricular pressures, local abnormalities of cardiac contraction, and intramural coronary artery abnormalities may lead to a chronical-ly ischemic state of the apical myocardium and to aneurysm formation.^2–4 Apical hypertrophy with aneurysm is frequently missed on echocardiography because of apical foreshortening and small apical cavity size; conversely, it is easily detected with use of CMR.