Paediatric Cardiology Department, Hospital de Santa Cruz, Lisbon, Portugal.
EuroIntervention. 2012 May 15;8(1):94-7. doi: 10.4244/EIJV8I1A15.
Patients with Ebstein's anomaly of the tricuspid valve may have right-to-left shunt at atrial level resulting in hypoxaemia, high haematocrit and hyperviscosity syndrome. The purpose of this study was to assess the results of percutaneous closure of atrial right-to-left shunt in patients with Ebstein's anomaly.
Records of patients treated between January 2002 and June 2010 were reviewed. Their condition before and after shunt closure (clinical data, oxygen saturation and haematocrit) were studied. During this period nine selected patients with Ebstein's anomaly and right-to-left shunt at atrial level were treated. Ages ranged from six to 67 years; seven were male. Mean pulmonary artery pressures were under 25 mmHg in all. Three patients had previous episodes of stroke and three had very high haematocrit, two of whom required therapeutic phlebotomies. Test occlusion of the shunt was performed in all patients with a balloon catheter, revealing an increase in systemic oxygen saturation, with right atrial pressures remaining <18 mmHg in all. Percutaneous closure of atrial shunt was achieved in all. There were no major complications. Arterial oxygen saturations increased in all patients from 85.0 ± 4.5% to 96.7 ± 1.5% (mean ± standard deviation). Medium follow-up was five years. The three patients with very high haematocrit levels had a decrease in its values from 62.9 ± 2.8% to 45.5 ± 3.9% after device occlusion. Both therapeutic phlebotomy programs were discontinued. All patients reported a marked improvement in effort tolerance.
Percutaneous closure of atrial right-to-left shunt in selected patients with Ebstein's anomaly offers significant improvement, abolishing hypoxaemia and hyperviscosity and preventing paradoxical embolisation.
三尖瓣下移畸形（Ebstein's anomaly）患者可能存在心房水平的右向左分流，导致低氧血症、高血球压积和高粘滞血症。本研究旨在评估经皮封堵三尖瓣下移畸形患者心房右向左分流的疗效。
回顾了 2002 年 1 月至 2010 年 6 月间接受治疗的患者记录。研究了分流封堵前后（临床数据、氧饱和度和血球压积）的情况。在此期间，治疗了 9 名选择的三尖瓣下移畸形合并心房水平右向左分流患者。年龄 6-67 岁，男性 7 名。所有患者肺动脉压均<25mmHg。3 例患者有脑卒中病史，3 例患者血球压积非常高，其中 2 例需要治疗性放血。所有患者均用球囊导管行分流封堵试验，发现全身氧饱和度增加，右心房压力均保持在<18mmHg。所有患者均成功完成了心房分流封堵，无重大并发症。所有患者的动脉血氧饱和度均从 85.0±4.5%增加到 96.7±1.5%（平均值±标准差）。中位随访时间为 5 年。3 例血球压积非常高的患者，封堵后血球压积值从 62.9±2.8%降至 45.5±3.9%。停止了 2 例患者的治疗性放血。所有患者均报告活动耐量明显改善。
在选择的三尖瓣下移畸形患者中，经皮封堵心房右向左分流可显著改善低氧血症和高粘滞血症，防止反常栓塞。
