Diagnosis and outcome in congenital ventricular diverticulum and aneurysm

Objective: True congenital ventricular diverticulum and aneurysm in children are very uncommon. We report our experience to clarify the diagnosis and outcome of these little-known entities. Methods: Twenty-two patients with congenital ventricular outpouchings were identified in our database from 1973 to 2004. Morphologic characteristics (localization, connection to a ventricle, contractility), histologic findings, and cardiac and/or extracardiac abnormalities were analyzed in all 22 patients. Cardiovascular events and clinical courses were reviewed. Results: Congenital ventricular diverticula (n = 16) were characterized by synchronal contractility and three myocardial layers on histologic examination. Two categories of congenital ventricular diverticulum could be identified with regard to their localization: apical and nonapical. Apical diverticula (n = 8) were always associated with midline thoracoabdominal defects and other heart malformations. Nonapical diverticula (n = 8) were always isolated defects. Congenital ventricular aneurysms (n = 6) were characterized by akinesis with paradoxical systolic motion, wide connection to the ventricle, fibrosis on histologic examination that appeared with high signal on T2 weighted magnetic resonance imaging, and absence of other heart or midline thoracoabdominal defects. The outcome was different in these two types of outpouchings: congenital ventricular aneurysms were associated with adverse outcomes whereas the prognosis for congenital ventricular diverticula was good. Conclusion: Congenital ventricular diverticulum and aneurysm are two distinct entities, with different histologic and morphologic characteristics and outcomes. Assessment of these differential characteristics is of importance for prenatal counseling.