Living donor liver transplantation in children with congenital heart disease.
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%.


CONCLUSIONS
LDLT is a safe procedure in a select group of ESLD patients with CHD.
Concejero
Allan
A
Liver Transplantation Program, Chang Gung Memorial Hospital-Kaohsiung Medical Center, and Chang Gung University College of Medicine, Taiwan.
Chen
Chao-Long
CL
Liang
Chi-Di
CD
Wang
Chih-Chi
CC
Wang
Shih-Ho
SH
Lin
Chih-Che
CC
Liu
Yeuh-Wei
YW
Yong
Chee-Chien
CC
Yang
Chin-Hsiang
CH
Lin
Tsan-Shiun
TS
Jawan
Bruno
B
Huang
Tung-Liang
TL
Cheng
Yu-Fan
YF
Eng
Hock-Liew
HL
D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000150:complications* / Q000151:congenital / Q000601:surgery*; D016031:Liver Transplantation / Q000009:adverse effects / Q000379:methods* / Q000502:physiology; D019520:Living Donors; D008297:Male; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome