Abstract

Congenital left ventricular (LV) aneurysm is a rare malformation of unknown cause that is often associated with a poor prognosis. This study was undertaken to evaluate the usefulness of cardiac magnetic resonance imaging in patients with congenital LV aneurysms and to determine the relation between clinical manifestations and the morphologic and functional characteristics of the aneurysms. Among the 26 consecutive patients with congenital LV aneurysms included, the anomalies involved the apex or free wall in 15 and the submitral myocardium in 11. Cardiac magnetic resonance provided detailed anatomic and functional assessment of the aneurysms in all patients. Compared with submitral aneurysms, apical or free-wall aneurysms were larger (24 +/- 29 vs 3 +/- 2 ml/m(2), p = 0.02), were more frequently associated with scar tissue by myocardial delayed enhancement imaging (71% vs 0%, p = 0.03), and tended to be more commonly associated with symptoms (53% vs 18%, p = 0.08). Aneurysm volume but not location correlated with LV size (r = 0.735, p <0.0001) and the ejection fraction (r = 0.774, p <0.0001). Apical or free-wall aneurysms were resected in 5 patients, with no mortality. There was 1 death after aortic valve replacement in a patient with type B Niemann-Pick disease, which was not clearly related to the LV aneurysm. The remaining 25 patients are alive at a median age of 13.5 years. In conclusion, the experience with this cohort illustrates that cardiac magnetic resonance is well suited for the morphologic and functional evaluation of congenital LV aneurysms.