Fattouh Aya M, Mogahed Engy A, Abdel Hamid Nehal, Sobhy Rodina, Saber Noha, El-Karaksy Hanaa
Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
National Research Center, Cairo, Egypt.
Arch Dis Child. 2016 Sep;101(9):803-7. doi: 10.1136/archdischild-2015-309589. Epub 2016 Apr 15.
There is deficiency of data about congenital heart defects (CHDs) in cholestatic disorders of infancy other than Alagille syndrome (AGS). We aimed to define the prevalence and types of CHDs in infants with various causes of cholestatic disorders of infancy.
This cross-sectional study was conducted on 139 infants presenting with cholestasis whether surgical or non-surgical. The study was carried out at the Pediatric Hepatology Unit, Cairo University Children's Hospital, Egypt. Full examination and investigations were done in an attempt to reach an aetiologic diagnosis for cholestasis, in addition to a comprehensive echocardiographic study.
The age at the onset of cholestasis ranged from 1 day to 7 months. Males constituted 61.2%. Biliary atresia (BA) was diagnosed in 39 patients (28%), AGS in 16 patients (11.5%), 27 patients had miscellaneous diagnoses and 57 cases had indeterminate aetiology. CHDs were detected in 55 patients (39.5%). Shunt lesions were detected in 24 patients (43.6%), pulmonary stenosis in 18 patients (32.7%) and combined lesions in 9 patients (16.4%). Three patients (5.5%) had abnormal cardiac situs. Only seven patients had clinical presentation suggestive of CHD. CHDs were detected in 14 patients with BA (35.9%), 15 patients with AGS (93.7%) and 26 patients in the remaining group (30.9%).
CHDs are not uncommon among cholestatic infants and are mostly asymptomatic. Echocardiographic examination of cholestatic infants is recommended particularly for patients with BA before undergoing hepatic portoenterostomy as presence of CHD may impact the anaesthetic planning and affect the outcome of hepatobiliary surgery.
除阿拉吉耶综合征（AGS）外，关于婴儿胆汁淤积性疾病中先天性心脏病（CHD）的数据不足。我们旨在确定婴儿期各种原因引起的胆汁淤积性疾病患儿中CHD的患病率和类型。
本横断面研究对139例出现胆汁淤积的婴儿进行，无论其是否接受手术治疗。该研究在埃及开罗大学儿童医院小儿肝病科进行。除了全面的超声心动图检查外，还进行了全面检查和调查，以试图对胆汁淤积做出病因诊断。
胆汁淤积开始时的年龄范围为1天至7个月。男性占61.2%。39例患者（28%）诊断为胆道闭锁（BA），16例患者（11.5%）诊断为AGS，27例患者有其他诊断，57例病因不明。55例患者（39.5%）检测出CHD。24例患者（43.6%）检测出分流性病变，18例患者（32.7%）检测出肺动脉狭窄，9例患者（16.4%）检测出合并病变。3例患者（5.5%）心脏位置异常。只有7例患者有提示CHD的临床表现。14例BA患者（35.9%）、15例AGS患者（93.7%）和其余组中的26例患者（30.9%）检测出CHD。
CHD在胆汁淤积婴儿中并不罕见，且大多无症状。建议对胆汁淤积婴儿进行超声心动图检查，特别是对BA患者在进行肝门肠吻合术前进行检查，因为CHD的存在可能影响麻醉计划并影响肝胆手术的结果。
