Regression of pulmonary arteriovenous malformations after transcatheter reconnection of the pulmonary arteries in patients with unidirectional Fontan.
Regression of pulmonary arteriovenous malformations after transcatheter reconnection of the pulmonary arteries in patients with unidirectional Fontan.
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DOI：
10.1111/j.1747-0803.2007.00094.x
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发表时间：
2007-05-01
影响因子：
0.3
通讯作者：
Moore, John
中科院分区：
文献类型：
--
作者：
AboulHosn, Jamil;Danon, Saar;Moore, John
目的：肺动静脉畸形(PAVM)发生于缺乏肝脏血流的肺。单向Fontan患者的上腔静脉(SVC)血流向左或右肺动脉(PA)，而下腔静脉(IVC)和肝脏血流向对侧PA。由于肺中的PAVM缺乏肝脏的血液，因此常会发生紫紫。我们报告了单向Fontan和PAVM患者经导管PA重建术的经验。设计：单向Fontan、发紫和PAVM患者接受了经导管PA重建术。在PAS同时注入造影剂后，用经间隔穿刺针将造影剂从一个PA穿刺入另一个PA。一种球囊安装的无覆盖支架被放置在连接肺动脉的桥上。该程序在PA之间提供了一种无限制的、永久的通信。对结果进行回顾性分析。结果：6例患者的平均年龄为14岁(13-35岁)。所有患者均为单肺功能心动过速，肺内有单向Fontan和PAVM，仅由SVC血流供血。所有病例均顺利完成再通手术，无并发症发生。6例患者平均随访时间为12.8个月(5.8~19.3个月)。术前平均血氧饱和度为0.86(0.81~0.92)，术后平均血氧饱和度为0.95(0.92~0.98)，平均提高9%(8~11%)。结论：PAS再通后，单向Fontan和PAVM患者血氧饱和度升高，提示PAVM消退。使用无覆盖支架可以安全地进行这一手术，并且它在改善全身血氧饱和度方面是有效的。
OBJECTIVE: Pulmonary arteriovenous malformations (PAVM) develop in patients with a lung deprived of hepatic blood flow. Unidirectional Fontan patients have superior vena cava (SVC) blood directed to the left or right pulmonary artery (PA), and inferior vena cava (IVC) and hepatic blood baffled to the contra-lateral PA. Cyanosis often develops due to PAVM in the lung deprived of hepatic blood. We report experiences with transcatheter PA reconnection in patients with unidirectional Fontan and PAVM.DESIGN: Patients with unidirectional Fontan, cyanosis, and PAVM underwent transcatheter PA reconnection. Following simultaneous injection of contrast into PAs, a transseptal needle was used to puncture from one PA to the other. A balloon-mounted, uncovered stent was positioned bridging the pulmonary arteries. The procedure provided a nonrestrictive, permanent communication between the PAs. Results were retrospectively reviewed. Catheterization data and oxygen saturations before and after procedure were analyzed.RESULTS: Six patients with a median age of 14 years (range 13-35 years) were identified. All patients had functional single ventricle with unidirectional Fontan and PAVM in the lung supplied solely by SVC flow. The reconnection procedure was performed successfully without complications in all cases. The median follow-up time for all 6 patients was 12.8 months (range 5.8-19.3 months). Median oxygen saturation was 0.86 (range 0.81-0.92) prior to the procedure, and 0.95 (range 0.92-0.98) on follow-up, with a median increase of 9% (range 8-11%).CONCLUSION: Patients with unidirectional Fontan and PAVM demonstrate increased oxygen saturations following reconnection of PAs, suggesting regression of PAVM. This procedure can be performed safely using uncovered stents, and it is effective in improving systemic oxygen saturations.