Abstract：
BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment.
METHODS: We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months.
CONCLUSIONS: Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.
METHODS: We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months.
CONCLUSIONS: Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.
摘要：
背景：先天性门体分流术（CPSS）是罕见的血管畸形，可分为肝外和肝内分流。肝外CPSS,也称为Abernethy畸形与严重的长期并发症，包括门性肺动脉高压，肝萎缩,高氨血症和肝性脑病。我们报告了迄今为止尚未描述的Abernethy畸形变体，需要采用创新的介入治疗方法。
方法：我们描述了一名31岁的患者，在6岁时进行了房室间隔缺损的手术修复。在长期随访中，他显示出持续的肺动脉高压，尽管进行了双重肺血管舒张治疗，但仍在恶化。当他出现动脉饱和度下降和有症状的高氨血症时，详细的重新评估显示了迄今为止尚未描述的连接门静脉和右下肺静脉的阿伯内西畸形的变异。在跨学科讨论之后，我们选择了介入方法。由于畸形无法通过顺行静脉或逆行动脉介入封堵，对门静脉进行了经肝穿刺.畸形的临时球囊闭塞显示门静脉压力仅略有增加。通过这种经肝途径，通过连续植入两个大血管闭塞装置，可以实现大血管连接的介入闭塞。介入后病程无明显变化，随访12个月时，氨水平和动脉饱和度均恢复正常。
结论：肺动脉高压或肺动静脉畸形的鉴别诊断应包括门静脉异常。基于对门静脉血流动力学的解剖和测试的仔细评估，可以在专业中心成功实施复杂的Abernethy畸形的介入治疗。
方法：我们描述了一名31岁的患者，在6岁时进行了房室间隔缺损的手术修复。在长期随访中，他显示出持续的肺动脉高压，尽管进行了双重肺血管舒张治疗，但仍在恶化。当他出现动脉饱和度下降和有症状的高氨血症时，详细的重新评估显示了迄今为止尚未描述的连接门静脉和右下肺静脉的阿伯内西畸形的变异。在跨学科讨论之后，我们选择了介入方法。由于畸形无法通过顺行静脉或逆行动脉介入封堵，对门静脉进行了经肝穿刺.畸形的临时球囊闭塞显示门静脉压力仅略有增加。通过这种经肝途径，通过连续植入两个大血管闭塞装置，可以实现大血管连接的介入闭塞。介入后病程无明显变化，随访12个月时，氨水平和动脉饱和度均恢复正常。
结论：肺动脉高压或肺动静脉畸形的鉴别诊断应包括门静脉异常。基于对门静脉血流动力学的解剖和测试的仔细评估，可以在专业中心成功实施复杂的Abernethy畸形的介入治疗。
