Akhtar Khurram, Sultan Mehboob, Ahmed Waqar, Ullah Maad, Sadiq Nadeem
Department of Paediatric Cardiology, Armed Forces Institute of Cardiology (AFIC) and National Institute of Heart Diseases (NIHD), Rawalpindi.
J Coll Physicians Surg Pak. 2014 May;24 Suppl 2:S129-31.
Pentalogy of Cantrell with ectopia cordis is a rare congenital anomaly, first described in 1958 by Cantrell, has a reported incidence of around 5-10 cases per one million live births with wide variety of clinical presentations. We are reporting a child with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus and diaphragmatic defect. Echocardiography in first month of life revealed a restrictive perimembranous ventricular septal defect and a small patent Foramen Ovale, both closed spontaneously in infancy. CT angiography at 10 months of age revealed a defect in the thoracic and abdominal walls along with herniation of left ventricular apex into epigastrium. The two ventriculi formed a tail that looked like a crocodile. This patient underwent surgical correction at our institution at 14 months of age and recovered well with no residual issue.
坎特雷尔五联症合并心脏异位是一种罕见的先天性异常，1958年由坎特雷尔首次描述，据报道每百万活产儿中约有5 - 10例发病，临床表现多样。我们报告一例患有心脏异位，同时伴有下胸骨裂、上腹壁缺损、脐异位和膈肌缺损的患儿。出生后第一个月的超声心动图显示为限制性膜周部室间隔缺损和一个小的卵圆孔未闭，两者在婴儿期均自发闭合。10个月大时的CT血管造影显示胸壁和腹壁有缺损，左心室心尖突入上腹部。两个心室形成一条尾巴，看起来像鳄鱼。该患者于14个月大时在我们机构接受了手术矫正，恢复良好，无残留问题。
