Living donor liver transplantation in children with congenital heart disease

Allan Concejero, Chao Long Chen*, Chi Di Liang, Chih Chi Wang, Shih Ho Wang, Chih Che Lin, Yeuh Wei Liu, Chee Chien Yong, Chin Hsiang Yang, Tsan Shiun Lin, Bruno Jawan, Tung Liang Huang, Yu Fan Cheng, Hock Liew Eng

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

12 Scopus citations

Abstract

BACKGROUND. The occurrence of congenital heart disease (CHD) with congenital biliary disease is uncommon. Our aim is to present our experience in living donor liver transplantation (LDLT) as treatment for end-stage liver disease (ESLD) in children with CHD. METHODS. A review of transplant records from June 1994 to December 2004 was performed. Twenty-three LDLT (13 males, 10 females) recipients were diagnosed to have both CHD and ESLD. RESULTS. CHD diagnoses were made preoperatively using transthoracic two-dimensional color flow Doppler echocardiography. The mean age was 22.3 months. There were 20 (87%) biliary atresia, two (9%) neonatal hepatitis, and one (4%) glycogen storage disease patients. Isolated CHD associated with ESLD included atrial septal defect (11, 48%), pulmonary stenosis (including 2 Alagille syndrome; 4, 17%), patent foramen ovale (4, 17%), ventricular septal defect (1, 4%), and mitral valve prolapse (1, 4%). Complex CHD included atrial septal defect + patent ductus arteriosus + patent foramen ovale (1, 4%), and atrial septal defect + pulmonary stenosis (1, 4%). The median Child's and Pediatric End-stage Liver Disease scores were 9, and 17, respectively. In all, 70% presented with varying degrees of pulmonary congestion pretransplant. There were no perioperative cardiac complications. Posttransplant, the patent foramen ovale in four recipients and atrial septal defect in four recipients closed spontaneously; and two recipients with pulmonary stenosis had their stenoses resolved spontaneously. The overall rejection rate was 17%. There was no mortality. The overall recipient and graft survivals at 1 and 5 years were both 100%. CONCLUSION. LDLT is a safe procedure in a select group of ESLD patients with CHD.

Original languageEnglish
Pages (from-to)484-489
Number of pages6
JournalTransplantation
Volume84
Issue number4
DOIs
StatePublished - 08 2007

Keywords

  • Biliary dysgenesis
  • Congenital cardiovascular malformation
  • Congenital heart disease
  • Liver disease
  • Liver transplantation

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