Pediatric Cardiovascular Surgery, Pediatric Cardiology, National Taiwan University Hospital, Taipei, Taiwan.
Int J Cardiol. 2011 Sep 15;151(3):313-7. doi: 10.1016/j.ijcard.2010.05.067. Epub 2010 Jul 1.
Uncorrected congenital heart defects (CHD) with severe pulmonary hypertension (sPH, systolic pulmonary artery>70% of systolic pressure) are usually considered inoperable. We are curious to know if some selected patients might benefit from palliative operation for those sPH with uncorrected CHD.
Adults or adolescents with sPH associated with ventricular septal defect (VSD) with/without great artery anomalies were selected for pulmonary artery banding (PAB) to reduce sPH. The target pulmonary pressure was less than half of the systolic blood pressure after arch or great arteries reconstruction. Repeated catheterization was performed to evaluate the feasibility of defect closure.
Consecutively, 8 patients (age 26 ± 9 years) received PAB as a palliative procedure in the past 8 years without mortality. The pre-PAB systolic pulmonary pressure was 119 ± 9 mmHg. Additional PAB had been applied in 4 of them. All patients showed significant improvement in function class (III to I or II). The mean post-PAB pulmonary pressure decreased significantly (77.5 ± 9.2 mmHg to 42.0 ± 9.0 mmHg) and 6-minute walk test was also found to have great improvement (270 ± 86 m to 414 ± 49 m), but the saturation at rest did not show a difference. Three of them received corrective surgery to close defects over 3-5 years.
For some selected adult sPH with uncorrected CHD, PAB can work as a palliative procedure to improve their functional class and even provide a chance of total repair.
未经矫正的先天性心脏病(CHD)合并严重肺动脉高压(sPH,收缩期肺动脉压>70%收缩压)通常被认为不能手术。我们很好奇,对于未经矫正的 CHD 合并 sPH 的某些选定患者,姑息性手术是否可能受益。
选择 sPH 合并室间隔缺损(VSD)伴/不伴大动脉异常的成人或青少年进行肺动脉带缩术(PAB)以降低 sPH。目标肺动脉压在大动脉或升主动脉重建后低于收缩压的一半。重复进行导管插入术以评估闭合缺损的可行性。
在过去 8 年中,连续有 8 例(年龄 26±9 岁)患者接受了姑息性 PAB 治疗,无死亡病例。PAB 前收缩期肺动脉压为 119±9mmHg。其中 4 例患者进行了额外的 PAB。所有患者的功能分级(III 级至 I 级或 II 级)均显著改善。平均肺动脉压在 PAB 后显著降低(77.5±9.2mmHg 至 42.0±9.0mmHg),6 分钟步行试验也发现有显著改善(270±86m 至 414±49m),但静息饱和度无差异。其中 3 例在 3-5 年内接受了矫正手术以闭合缺损。
对于某些选定的成人 sPH 合并未经矫正的 CHD,PAB 可以作为姑息性治疗方法,改善其功能分级,甚至提供完全修复的机会。