백재숙¹, 제현곤¹ ,권보상¹ ,김기범¹ ,배은정¹ ,노정일¹ ,윤용수¹ ,최정연¹ ,박은아² ,이활² |
Background: Although late liver dysfunction after the Fontan operation has been identified, the nature of this potentially serious complication has not been understood.
Objective: To identify the prevalence, clinical characteristics, and risk factors of hepatic complication after Fontan operation.
Methods: This is the cross sectional study on 98 Fontan patients who performed cardiac CT, among 199 late follow-up patients, having undergone the Fontan procedure between 1986 and 2003. We reviewed clinical features, echocardiogram, radiologic features, and biochemical test. The mean age was 18.4±6.years. The mean duration since the initial Fontan operation was 11.9±4.5 years.
Result: Sixty eight patients (20.0±5.7years old, male:female=41:27) had hepatic complications, including radiologic features of liver cirrhosis(36.7%), thrombocytopenia(21.5%) due to splenomagaly, hyperbilirubinemia(44.9%) and hepatic masses (2%). The mean duration since the initial Fontan operation was 14.6±5.0 years. Hepatic complications occurred more frequently in patients with longer follow-up duration(p<0.001). Hepatic complications were associated significantly with decreased ventricular function(p=0.05) and increased ventricular Tei index.(p<0.001). The morphology of single ventricle, early postoperative hospital stay, sex, degree of valve regurgitation, and sinus node dysfunction were not significantly correlated with hepatic complication.
Conclusion: Late hepatic dysfunction and cirrhotic change were frequent in Fontan palliated patients. Hepatic complications were related to duration of Fontan circulation and ventricular dysfunction. After Fontan operation, regular evaluation of hepatic condition is required.
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