Diagnosis and outcome in congenital ventricular diverticulum and aneurysm
Diagnosis and outcome in congenital ventricular diverticulum and aneurysm
复制标题
DOI：
10.1016/j.jtcvs.2005.09.046
复制
发表时间：
2006-02-01
影响因子：
6
通讯作者：
Bonnet, D
中科院分区：
文献类型：
--
作者：
Marijon, E;Ou, P;Bonnet, D
目的：小儿先天性室性憩室及动脉瘤十分少见。我们报告我们的经验，以澄清这些鲜为人知的实体的诊断和结果。方法：对1973 ~ 2004年22例先天性脑室外凸患者进行分析。对所有22例患者的形态学特征（定位、与心室的连接、收缩性）、组织学表现以及心脏和/或心外异常进行分析。回顾了心血管事件和临床病程。结果：先天性心室憩室16例，组织学检查表现为心肌同步收缩，心肌有三层。先天性心室憩室的定位可分为两类：根尖型和非根尖型。根尖憩室（n = 8）常伴有胸腹中线缺损及其他心脏畸形。非根尖憩室（n = 8）均为孤立性缺陷。先天性心室动脉瘤（n = 6）表现为收缩运动异常，与心室连接较宽，病理检查呈纤维化，T2加权磁共振成像显示高信号，无其他心脏或胸腹中线缺损。这两种类型的结果是不同的：先天性脑室动脉瘤的预后不良，而先天性脑室憩室的预后良好。结论：先天性室性憩室与动脉瘤是两种不同的实体，具有不同的组织学、形态学特征和预后。评估这些差异特征对产前咨询很重要。
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.