Liver transplantation in an adult patient with hepatocellular carcinoma following liver cirrhosis as a complication of the Fontan procedure -A case report-
조민경, 권지혜, 곽미숙, 조재원, 황지윤, 김갑수,
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조민경 ( Cho Min-Kyung ) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Anesthesiology and Pain Medicine
권지혜 ( Kwon Ji-Hye ) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Anesthesiology and Pain Medicine
곽미숙 ( Gwak Mi-Sook ) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Anesthesiology and Pain Medicine
조재원 ( Joh Jae-Won ) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Surgery
황지윤 ( Hwang Ji-Yun ) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Anesthesiology and Pain Medicine
김갑수 ( Kim Gaab-Soo ) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Anesthesiology and Pain Medicine
Abstract
Background: Fontan-associated liver disease (FALD) is a hepatic disorder caused by hemodynamic changes and systemic venous congestion following the Fontan procedure. FALD includes liver cirrhosis and hepatocellular carcinoma (HCC), both of which may require liver transplantation (LT). However, the Fontan circulation, characterized by elevated central venous pressure and reduced cardiac output, is a challenging issue for surgeons and anesthesiologists.
Case: We report a living-donor LT for the treatment of HCC. The patient was a 24-year-old male who underwent the Fontan procedure for pulmonary atresia and right ventricle hypoplasia. We focused on maintaining enough blood volume for cardiac output without causing pulmonary edema, as the patient is not well adapted to changes in volume. Owing to a multidisciplinary approach, the surgery was successfully performed without fatal adverse events.
Conclusions: To our knowledge, this is the first case of isolated LT in a recipient who became an adult after having undergone the Fontan procedure.
키워드
Fontan procedure; Hepatocellular carcinoma; Liver cirrhosis; Liver transplantation
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