Background: Congenital left ventricular aneurysm is a poorly understood and potentially lethal entity. Methods and Results In a clinicopathologic study of 7 new cases, the major presenting features in 6 patients were congestive heart failure in 4, ventricular arrhythmias in a 32-week fetus, and multiple congenital anomalies in a fetus with trisomy 13. Accurate diagnosis was achieved in all 3 living patients by echocardiography, angiocardiography, and magnetic resonance imaging. The aneurysm was predominantly apical in 3 and involved most of the left ventricular free wall in 4. Of the 3 living patients, medical management alone sufficed in 2. The third, a newborn boy, underwent a new and successful aneurysm-exclusion left ventriculoplasty. The mitral valve was abnormal in all 4 autopsied cases, the papillary muscles being short, thin, or absent. The aneurysm was thinner and its area was larger than that of the nonaneurysmal left ventricle in all necropsied patients.
Conclusions: Congenital left ventricular aneurysm appears to be a developmental anomaly, an idiopathic dysplasia of left ventricular endocardium and myocardium. No evidence of a viral etiology was found. Some neonates can be managed medically, but others require urgent surgical intervention. A new surgical operation is presented, a functional left ventricular aneurysmectomy that minimizes intraoperative and postoperative blood loss and that preserves the coronary arteries.