Zani-Ruttenstock Elke, Zani Augusto, Honjo Osami, Chiu Priscilla
Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.
Division of Cardiovascular Surgery, Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, Ontario, Canada.
European J Pediatr Surg Rep. 2017 Jan;5(1):e9-e11. doi: 10.1055/s-0037-1599794.
Pentalogy of Cantrell is a rare syndrome that is characterized by varying degrees of midline wall defects and congenital cardiac anomalies. A left ventricular diverticulum (LVD) is defined as partial ectopia cordis, can be part of the pentalogy of Cantrell, and can put the patient at risk of severe complications. Early diagnosis and ligation/resection of the LVD is important to prevent complications. We report on a case of pentalogy of Cantrell, in which a LVD was diagnosed only at 2 months of age despite preceding pre- and postnatal echocardiography. We conclude that in the suspicion of pentalogy of Cantrell, either complete or incomplete, cardiac magnetic resonance imaging should be performed, when possible, to avoid the potential complication of a herniating ventricular diverticulum.
坎特雷尔五联症是一种罕见综合征，其特征为不同程度的中线壁缺损和先天性心脏异常。左心室憩室（LVD）被定义为部分心脏异位，可为坎特雷尔五联症的一部分，并可使患者面临严重并发症风险。早期诊断并结扎/切除LVD对于预防并发症很重要。我们报告一例坎特雷尔五联症病例，尽管产前和产后均进行了超声心动图检查，但该例患者直到2个月大时才诊断出LVD。我们得出结论，在怀疑存在完全或不完全性坎特雷尔五联症时，应尽可能进行心脏磁共振成像，以避免心室憩室疝出的潜在并发症。
