Childhood cirrhosis, hepatopulmonary syndrome and liver transplantation.
OBJECTIVE
The hepatopulmonary syndrome (HPS) is characterized as a triad: liver disease, intrapulmonary vascular dilatation, and arterial hypoxemia. The aim of this study is to analyze outcome of children with HPS in liver transplant era.


METHODS
Between September 1996 and November 2006, 172 cirrhotic patients (median age 5 years; range 0.2-22 years, M/F; 97/75) were followed at Ege University Pediatric Gastroenterology, Hepatology and Nutrition Unit. All patients were evaluated by chest radiography, arterial blood gas analysis, and alveolar-arterial oxygen tension difference, contrast echocardiography (CEE) after and before the liver transplantation.


RESULTS
HPS was diagnosed in 33 patients (19%) by CEE. None of them had pulmonary hypertension. HPS was not found related to etiology of the liver disease. Portal hypertension was found related to the development of HPS (75.7% in patients with HPS and 54.6% in others, p = 0.02). 17 of 33 patients with HPS underwent liver transplantation. Preoperative and postoperative period of these patients was uneventful. Patients were extubated in the operating room except for two. Median follow up of transplanted children was 1.9 year (range; 0.75-10 years). Arterial blood gas analysis and CEE positivity regressed in all of them by postoperative 6th month.


CONCLUSIONS
HPS is a serious and important complication of cirrhotic children that leads to tissue hypoxia and central cyanosis. HPS seems reversible after liver transplantation in all patients.
Tumgor
Gokhan
G
Department of Pediatric, Division of Gastroenterology, Hepatology and Nutrition, Ege University School of Medicine Organ Transplantation and Research Center, Izmir, Turkey. gtumgor74@yahoo.com
Arikan
Cigdem
C
Yuksekkaya
Hasan Ali
HA
Cakir
Murat
M
Levent
Erturk
E
Yagci
Rasit Vural
RV
Kilic
Murat
M
Aydogdu
Sema
S
D000293:Adolescent; D000328:Adult; D001784:Blood Gas Analysis; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D020065:Hepatopulmonary Syndrome / Q000150:complications* / Q000601:surgery / Q000628:therapy*; D006801:Humans; D007223:Infant; D008103:Liver Cirrhosis / Q000150:complications* / Q000601:surgery / Q000628:therapy*; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D016896:Treatment Outcome