Congenital left ventricular aneurysm: Clinical, imaging, pathologic, and surgical findings in seven new cases

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, ventr icular arrhythmias in a 32-week fetus, and multiple congenital anomalies in a fetus with trisomy 13. Accurate diagnosis was achieved in all 3 living p atients by echocardiography, angiocardiography, and magnetic resonance imag ing. The aneurysm was predominantly apical in 3 and involved most of the le ft 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 success ful aneurysm-exclusion left ventriculoplasty. The mitral valve was abnormal in all 4 autopsied cases, the papillary muscles being short, thin, or abse nt. The aneurysm was thinner and its area was larger than that of the nonan eurysmal left ventricle in all necropsied patients. Conclusions Congenital left ventricular oneurysm appears to be a developmen tal anomaly, on idiopathic dysplasia of left ventricular endocardium and my ocardium. No evidence of a viral etiology was found. Some neonates can be m anaged medically, but others require urgent surgical intervention. A new su rgical operation is presented, a functional left ventricular aneurysmectomy that minimizes intraoperative and postoperative blood loss and that preser ves the coronary arteries.