Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension
Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension
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DOI：
10.1016/j.ijcard.2007.08.016
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发表时间：
2008-10-13
影响因子：
3.5
通讯作者：
Veldtman, Gruschen R.
中科院分区：
文献类型：
--
作者：
Lim, Zek S.;Vettukattill, Joseph J.;Veldtman, Gruschen R.
背景：复杂性肺动脉闭锁（CPA），即肺动脉闭锁伴室间隔缺损和主肺动脉侧支循环（MAPCA）或法洛四联症合并 MAPCA，其临床病程经常并发肺动脉（PA）高血压。方法：对 16 岁以上的 CPA 或法洛四联症合并 MAPCA 和 PA 高血压患者进行西地那非治疗。回顾了病例记录的基线和随访（西地那非后）特征。结果：确定了 5 名患者，其中 4 名女性，中位年龄 28 岁（范围 18 至 47）岁。所有患者的症状均得到改善：4 名坐轮椅的患者中有 2 名反应显着，在接受西地那非治疗后 6 分钟内分别行走了 345 米和 157 米。 4 名患有明显 PA 树枝状异常和严重心室功能障碍的患者中，有 1 名患者的症状初步得到改善。另一名患者则从步行不到 100 m 改善到爬了 2 层楼梯。 2 例患者的动脉饱和度分别从 70% 和 60% 提高到 87% 和 84%，而 1 例患者的动脉饱和度保持不变，尽管进行了经典 Blalock-Taussig 分流术的栓塞。一名 PA 树枝化/PA 床位减少的患者无法耐受西地那非。 结论：西地那非耐受性良好，当单独使用或作为经皮 PA 血管成形术的辅助治疗时，西地那非可改善大多数患有 PA 高血压的 CPA 患者的症状并改善饱和度。 (C) 2007 Elsevier Ireland Ltd. 保留所有权利。
Background: Complex pulmonary atresia (CPA) i.e. pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals (MAPCAs) or Tetralogy of Fallot with MAPCAs frequently have a clinical course complicated by development of pulmonary arterial (PA) hypertension.Methods: A cross-sectional retrospective review of patients >16 years with CPA or Tetralogy of Fallot with MAPCAs and PA hypertension treated with sildenafil was conducted. Case notes were reviewed for baseline and follow-up (after sildenafil) characteristics.Results: Five patients, 4 female, median age 28 (range 18 to 47) years, were identified. All patients experienced symptomatic improvement: 2 of 4 wheelchair bound patients responded dramatically and walked 345 and 157 m respectively in 6 min following sildenafil therapy. One of the 4 with marked PA arborization abnormalities and severe ventricular dysfunction had initial symptomatic improvement. Another patient improved from walking less 100 m to climbing 2 flights of stairs. Arterial saturations improved in 2 cases from 70 and 60% to 87 and 84% respectively, whilst arterial saturations remained static in 1 case despite embolization of a classical Blalock-Taussig shunt. One patient with PA arborization/diminished PA bed was unable to tolerate sildenafil.Conclusions: Sildenafil is well tolerated and leads to symptomatic improvement and better saturations in the majority of patients with CPA with PA hypertension when used in isolation or as an adjunct to percutaneous PA angioplasty. (C) 2007 Elsevier Ireland Ltd. All rights reserved.