Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation
Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation
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DOI：
10.1111/j.1432-2277.2009.00964.x
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发表时间：
2010-01-01
影响因子：
3.1
通讯作者：
Uemoto, Shinji
中科院分区：
文献类型：
--
作者：
Iida, Taku;Ogura, Yasuhiro;Uemoto, Shinji
在本报告中，我们描述了1例患有阿伯内西2型先天性肝外门腔分流（CEPS）的患者（7岁男孩）的活体肝移植（LDLT）情况。该患者首次治疗肺动脉高压时接受了外科分流结扎术；首次手术后4年，肺动脉高压得到改善并成功控制。然而，由于存在多处肝内门体分流，肺动脉高压逐渐复发；因此，将活体肝移植作为肝内门体分流的根治性治疗方法。在活体肝移植22个月后，他的肺动脉压也得到控制，术后持续静脉注射前列腺素I₂（PGI₂）治疗得以成功停用。我们建议临床医生仔细随访阿伯内西2型先天性肝外门腔分流继发的门体分流复发和心肺疾病。
P>In this report, we describe a living donor liver transplantation (LDLT) in a patient (7-year-old boy) with Abernethy type 2 congenital extrahepatic portocaval shunts (CEPS). This patient underwent a surgical shunt ligation as the first treatment for pulmonary hypertension; pulmonary hypertension was improved and controlled successfully 4 years after the first operation. However, pulmonary hypertension recurred gradually because of multiple intrahepatic portosystemic shunts; therefore, LDLT was performed as a radical treatment of intrahepatic portosystemic shunts. His pulmonary arterial pressure was also controlled 22 months after LDLT, the postoperative continuous intravenous prostaglandin I(2) (PGI(2)) treatment could be withdrawn successfully. We suggest that clinicians carefully follow up the recurrent portosystemic shunt and cardiopulmonary disorders secondary to Abernethy type 2 CEPS.