title: The prevalence of congenital heart defects in infants with cholestatic disorders of infancy: a single-centre study.
abstract: 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.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
authors.forename: Aya M
authors.initials: AM
authors.lastname: Fattouh
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
authors.forename: Engy A
authors.initials: EA
authors.lastname: Mogahed
authors.affiliation: National Research Center, Cairo, Egypt.
authors.forename: Nehal
authors.initials: N
authors.lastname: Abdel Hamid
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
authors.forename: Rodina
authors.initials: R
authors.lastname: Sobhy
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
authors.forename: Noha
authors.initials: N
authors.lastname: Saber
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
authors.forename: Hanaa
authors.initials: H
authors.lastname: El-Karaksy
mesh_terms: D016738:Alagille Syndrome / Q000453:epidemiology; D001656:Biliary Atresia / Q000150:complications / Q000453:epidemiology; D002779:Cholestasis / Q000453:epidemiology* / Q000209:etiology; D003430:Cross-Sectional Studies; D004452:Echocardiography; D004534:Egypt / Q000453:epidemiology; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000453:epidemiology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015995:Prevalence
keywords: Jaundice; Metabolic